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Intravascular large B-cell lymphoma (IVL) is a rare type of lymphoma characterized by tumor growth selectively within the vessels. The 5th edition of the World Health Organization classification defines IVL as a large B-cell lymphoma, the same as diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS). Since the clinical manifestations of IVL are nonspecific, the diagnosis is time-consuming, and the course is often fatal. Serum soluble interleukin-2 receptor (sIL-2R) and serum lactate dehydrogenase (LDH) levels are known to be elevated in a variety of lymphomas. However, the mechanism of sIL-2R elevation in B-cell lymphomas is not fully understood. In this study, we analyzed the serum level of laboratory findings, including sIL-2R and LDH, as well as the presence of B symptoms in 39 patients with IVL, and compared them with 56 patients with stage IV DLBCL. Both sIL-2R and LDH levels were significantly higher in IVL than in DLBCL (p = 0.035 andp = 0.002, respectively). In IVL, there were no significant differences in both sIL-2R and LDH levels between patients with and without B symptoms (p = 0.206 andp = 0.441, respectively). However, in DLBCL, both sIL-2R and LDH levels were significantly higher in the presence of B symptoms (p = 0.001 andp < 0.001, respectively). The high sIL-2R and LDH levels in IVL may be related to the peripheral blood microenvironment, but further studies are needed to verify this.

Nodal marginal zone lymphoma (NMZL) is a type of low-grade B-cell lymphoma arising in the follicular marginal zone of the lymph nodes. It is an extremely rare disease that accounts for 1.5 %–1.8 % of all malignant lymphomas. We herein report a case of nodal marginal zone lymphoma arising in the submental region. A 45-year-old woman was referred to our hospital because of swelling of the submental region. Contrast-enhanced computed tomography revealed 2 masses measuring 25 and 10 mm in diameter in the submental region, and the inside of the masses showed uniform enhancement. Magnetic resonance imaging revealed a low signal intensity on T1-weighted imaging and high signal intensity on T2-weighted images. Given these findings, the clinical diagnosis was submental tumor. We performed lymphadenectomy under general anesthesia. The histopathological diagnosis was NMZL. Subsequently, the patient was referred to the Department of Hematology, and a general examination was performed. There were no B symptoms, such as a fever or weight loss, and no lesions were found at other sites. The patient was ultimately diagnosed with Stage IA disease by Ann Arbor staging. At present, 26 months have passed, and the postoperative course has been uneventful.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare, cutaneous lymphoma involving subcutaneous adipose tissue. SPTL is associated in less than 20% with hemophagocytic syndrome (HPS). A 5-year overall survival rate is inferior in patients with SPTL and HPS (46%) as compared with 91% in patients without HPS. No standardized therapy for SPTCL has yet been established. This is a case of 35-year-old Caucasian man with a one-month history of B symptoms with the suspicion of Still's disease, at admission with leucopenia, high LDH, ferritin, sIl-R2, and triglycerides levels, hepatosplenomegaly, small right supraclavicular nodule, and irregular thickening of trunk subcutaneous tissue. The abdomen MRI showed generalized thickening of mesentery and colonic mucosa. In the patient, diagnosis of SPTCL was established with secondary HPS. CHOEP chemotherapy and modified HLH 2014 protocol were applied with subsequent high dose chemotherapy (BEAM) supported by autologous stem cells transplantation. Treatment was complicated by pancytopenia and pneumonia. The outcome of the disease treated by intensive protocol seems to be good.

Diffuse large B-cell lymphoma (DLBCL) is an aggressive and rapid-growing form of non-Hodgkin lymphoma (NHL). The objective of this research was to assess the predictive role of lymphocyte to monocyte ratio (LMR), red cell distribution width (RDW) and neutrophil to lymphocyte ratio (NLR) values in the survival of DLBCL patients. A retrospective analysis of 136 DLBCL patients admitted to Nanakali Hospital for blood diseases and oncology from 2010-2020 was done. We assessed the correlation of LMR, RDW and NLR with patients’ characteristics and the impact on survival by the Kaplan–Meier method, the log-rank test, and Cox regression models for multivariate analysis. The complete remission rate was 61.7%, with a 5- year overall survival (OS) and progression-free survival (PFS) of 59.5% and 60%, respectively. The Log-rank test showed that LMR was significantly correlated with Ann Arbor staging (p= 0.040). There is a significant association between RDW and Eastern Cooperative Oncology Group performance status (ECOG-performance status) (p= 0.022), B symptoms (p= 0.026), Revised International prognostic index (R-IPI) (p= 0.004), lactate dehydrogenase (LDH) (p= 0.021), and beta 2 microglobulin (B2MG) (p= 0.007), whereas NLR had a significant correlation with LDH only (p=0.016). There were no significant differences in the 5-year OS or PFS in patients with different levels of RDW, LMR, and NLR. LMR, RDW and NLR were correlated with many of patients’ characteristics. However, none of the LMR, RDW and NLR did possess value to predict OS and PFS, and they cannot be used as biomarkers for survival evaluation of DLBCL.

Background Older adults with Hodgkin Lymphoma (HL) have poorer outcomes than younger patients. There are little data about which baseline patient and disease factors inform prognosis among older patients. We sought to create a prediction model for 1-year mortality among older patients with new HL who received dose-intense chemotherapy. Methods We included adults ≥65 years old with a new diagnosis of classical HL between 2000–2013 from the Surveillance, Epidemiology, and End Results (SEER)-Medicare dataset who received full-regimen chemotherapy. Through a non-random 2:1 split, we created development and validation cohorts. Multiple imputation was used for missing data. Using stepwise selection and logistic regression, we identified predictive variables for 1-year mortality. The model was applied to the validation cohort. A final model was then fit in the full cohort. Results We included 1315 patients. In the development cohort (n = 813), we identified significant predictors of 1-year mortality including age, Charlson comorbidity index (CCI), B symptoms at diagnosis, and advanced stage at diagnosis. The c-statistic was 0.70. When this model was applied to the validation cohort (n = 502), the c-statistic was 0.65. Predictors of 1-year mortality in the final model were CCI (OR = 1.41), B symptoms (OR = 1.54), advanced stage (OR = 1.44), and older age at diagnosis (OR = 1.33). Conclusion We present a prediction model for use among older adults with HL who receive intensive chemotherapy. We identify risk factors for death within 1 year of diagnosis. Future work will build upon prognostication and shared decision-making after diagnosis for this population.

Background: Primary adrenal (PA) diffuse large B cell lymphoma (DLBCL) was previously reported as an aggressive subset of DLBCL, but its genetic features were not sufficiently characterized. From our previous study of DLBCL with programmed death-ligand 1 (PD-L1) gene alterations, we focused on PD-L1 gene alterations in PA-DLBCL with clinicopathologic implications. Methods: We performed fluorescence in situ hybridization for PD-L1 gene translocation and amplification in PA-DLBCL (n = 18) and comparatively analyzed clinicopathologic characteristics with systemic non-adrenal (NA)-DLBCL (n = 90). Results: PA-DLBCL harbored distinctive features (vs. NADLBCL), including high international prognostic index score (3–5) (72% [13/18] vs. 38% [34/90], p = .007), poor Eastern Cooperative Oncology Group performance score (≥ 2) (47% [7/15] vs. 11% [10/90], p = .003), elevated serum lactate dehydrogenase (LDH) (78% [14/18] vs. 51% [44/87], p = .035) and MUM1 expression (87% [13/15] vs. 60% [54/90], p = .047). Moreover, PA-DLBCL showed frequent PD-L1 gene alterations (vs. NA-DLBCL) (39% [7/18] vs. 6% [5/86], p = .001), including translocation (22% [4/18] vs. 3% [3/87], p = .016) and amplification (17% [3/18] vs. 2% [2/87], p = .034). Within the PA-DLBCL group, PD-L1 gene–altered cases (vs. non-altered cases) tended to have B symptoms (p = .145) and elevated LDH (p = .119) but less frequent bulky disease (≥ 10 cm) (p = .119). In the survival analysis, PA-DLBCL had a poor prognosis for overall survival (OS) and progression-free survival (PFS) (vs. NA-DLBCL; p = .014 and p = .004). Within the PA-DLBCL group, PD-L1 translocation was associated with shorter OS and PFS (p < .001 and p = .012). Conclusions: PA-DLBCL is a clinically aggressive and distinct subset of DLBCL with frequent PD-L1 gene alterations. PD-L1 gene translocation was associated with poor prognosis in PA-DLBCL.

Objective: To identify the prognostic factors in relapsed Hodgkin’s Lymphoma patients with regards to their impact on the outcome of autologous hematopoietic stem cell transplant. Study Design: Retrospective observational study. Place and Duration of Study: Department of Medical Oncology, Shaukat Khanum Memorial Cancer Hospital & Research Center, Lahore, Jun 1999 to Jun 2019. Methodology: Out of a total of 2061 Hodgkin’s Lymphoma patients, 37 (1.8%) patients with relapsed disease underwent autotransplant and were studied using the Hospital Information System. We obtained details of clinicopathological factors, treatment, and outcome. In our study, the outcome variable was event after transplant (relapse/disease progression/death). Results: Among the 37 relapsed Hodgkin’s Lymphoma patients undergoing auto-transplant, 24 (64.9%) patients had an early relapse after first-line chemotherapy. In this early relapse group of 24 patients, 9 (37.5%) remained well after auto-transplant but 15 (62.5%) patients had an event. Out of 37 relapsed Hodgkin’s Lymphoma patients undergoing auto-transplant, a complete response on pre-transplant imaging was observed in 24 (64.9%) patients. In those 24 patients with a complete response on pre-transplant imaging, 16 (66.7%) patients remained well after transplant while 8 (33.3%) patients had a worse event. Twoyear progression-free and overall survival proportions were 56% and 77% respectively. Conclusion: Complete metabolic response on pre-transplant imaging was associated with better overall survival. Adverse factors observed were initial short duration of complete response, bulky disease at relapse, variables comprising international prognostic score, B symptoms, and raised erythrocyte sedimentation rate at relapse.

Follicular lymphoma is clinically classified as a common type of indolent non-Hodgkin's lymphoma, and its clinical diagnosis is difficult because B symptoms and elevated soluble interleukin-2 receptor (sIL-2R) levels are less frequent in follicular lymphoma than in other lymphomas. We report a case of follicular lymphoma masquerading immunoglobulin G4-related disease (IgG4-RD) with elevated IgG4 levels. A 67-year-old man presented to our hospital with a 1-year history of deep right supraclavicular and para-aortic lymph node lymphadenopathy on plain computed tomography (CT) findings along with elevated IgG4 levels, and the 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) scan showed heterogeneous diffuse FDG uptake in the liver, and FDG uptake was noted at multiple sites in the enlarged right supraclavicular and para-aortic lymph nodes. Excisional biopsy of the right supraclavicular lymph node, performed under general anesthesia, showed a tumor-like structure mimicking a normal germinal center in the lymphoid follicle; immunostaining was positive for B-cell lymphoma 2 and CD10 proteins with some plasma cells stained with IgG, only 30% of them were positive for IgG4, and no marked fibrosis characteristic of IgG4-RD was observed; therefore, follicular lymphoma was diagnosed, and all symptoms, including FDG uptake, improved with rituximab monotherapy. Differential diagnoses of slowly progressive generalized lymphadenopathy over the years with elevated serum IgG4 levels include IgG4-RD, Castleman's disease, and indolent lymphoma. Multiple accumulation in the liver on FDG-PET/CT, if found, may suggest indolent lymphoma among them.

Background Primary pancreatic lymphoma (PPL) is a rare neoplasm. Being able to distinguish it from other pancreatic malignancies such as pancreatic ductal adenocarcinoma (PDAC) is important for appropriate management. Unlike PDAC, PPL is highly sensitive to chemotherapy and usually does not require surgery. Therefore, being able to identify PPL preoperatively will not only direct physicians towards the correct avenue of treatment, it will also avoid unnecessary surgical intervention. Aim To evaluate the typical and atypical multi-phasic computed tomography (CT) imaging features of PPL. Methods A retrospective review was conducted of the clinical, radiological, and pathological records of all subjects with pathologically proven PPL who presented to our institutions between January 2000 and December 2020. Institutional review board approval was obtained for this investigation. The collected data were analyzed for subject demographics, clinical presentation, laboratory values, CT imaging features, and the treatment received. Presence of all CT imaging findings including size, site, morphology and imaging characteristics of PPL such as the presence or absence of nodal, vascular and ductal involvement in these subjects were recorded. Only those subjects who had a pre-treatment multiphasic CT of the abdomen were included in the study. Results Twenty-nine cases of PPL were diagnosed between January 2000 and December 2020 (mean age 66 years; 13 males/16 females). All twenty-nine subjects were symptomatic but only 4 of the 29 subjects (14%) had B symptoms. Obstructive jaundice occurred in 24% of subjects. Elevated lactate dehydrogenase was seen in 81% of cases, whereas elevated cancer antigen 19-9 levels were present in only 10% of cases for which levels were recorded. The vast majority (90%) of tumors involved the pancreatic head and uncinate process. Mean tumor size was 7.8 cm (range, 4.0-13.8 cm). PPL presented homogenous hypoenhancement on CT in 72% of cases. Small volume peripancreatic lymphadenopathy was seen in 28% of subjects. Tumors demonstrated encasement of superior mesenteric vessels in 69% of cases but vascular stenosis or occlusion only manifested in 5 out of the twenty-nine individuals (17%). Mild pancreatic duct dilatation was also infrequent and seen in only 17% of cases, whereas common bile duct (CBD) dilation was seen in 41% of subjects. Necrosis occurred in 10% of cases. Size did not impact the prevalence of pancreatic and CBD dilation, necrosis, or mesenteric root infiltration (P = 0.525, P = 0.294, P = 0.543, and P = 0.097, respectively). Pancreatic atrophy was not present in any of the subjects. Conclusion PPL is an uncommon diagnosis best made preoperatively to avoid unnecessary surgery and ensure adequate treatment. In addition to the typical CT findings of PPL, such as homogeneous hypoenhancement, absence of vascular stenosis and occlusion despite encasement, and peripancreatic lymphadenopathy, this study highlighted many less typical findings, including small volume necrosis and pancreatic and bile duct dilation.

Objective Diffuse Large B Cell Lymphoma (DLBCL) is the most common type of Non-Hodgkin Lymphoma (NHL). The aim of this study was to assess the clinico pathological characteristics of DLBCL specifically, among the affected individuals residing in Northern areas of Pakistan who had not been previously included in major lymphoma studies due to their remote location. Results Mean age of the patients was 49.7 years. Male: female ratio was 1.5:1. Primary site was lymph node in 99 (71.74%) patients, out of which, 36 (26.09%) patients had B symptoms and 19 (13.77%) patients had stage IV disease. 39 (28.26%) patients had primary extra nodal involvement, 4 (2.90%) patients had B symptoms and 3 (2.17%) had stage IV disease. Extra nodal sites involved in primary extra nodal DLBCL were gastrointestinal tract (GIT) 19 (48.72%), tonsils 6 (15.38%), spine 4 (10.26%), soft tissue swelling 3 (7.69%), parotid gland 2 (5.13%), thyroid 2 (5.13%) central nervous system (CNS) 1 (2.56), breast 1 (2.56%) and bone marrow 1 (2.56%). Our study revealed increased percentage of patients with nodal DLBCL in stage IV and with B symptoms. Few patients with primary extra nodal DLBCL had B symptoms and stage IV disease at presentation. GIT was the most common site of involvement in primary extra nodal DLBCL.

Introduction Most Indian centers use Adriamycin/Bleomycin/Vinblastine/Dacarba-zine (ABVD) chemotherapy for pediatric Hodgkin lymphoma (pHL). To reduce the late toxicity, robust predictive markers are needed to risk stratify pHL patients, thereby limiting the number of chemotherapy cycles and omitting radiation for low-risk and intensifying treatment for high-risk children. Objective This study was conducted to analyze the outcome of pHL patients treated with ABVD and various factors predicting the outcome. Materials and Methods This retrospective study analyzed the outcome of 113 consecutive pHL children treated with ABVD chemotherapy from 11 tertiary care centers in South India from 2009 to 2019. Results The median duration of follow-up was 2.73 years. The median age was 13 years. B symptoms are seen in 50.5% patients, bulky disease in 23%, and stage IV in 28.3%. Of 113 pHL, 69% had a positron emission tomography (PET) and 31% had computed tomography (CT)-based staging. Stage IV (37.1%) and extranodal involvement (31.2%) were seen more often with PET than with CT staging (8.5 and 2.8%, respectively). Among 64 patients with interim PET scan after two cycles (iPET2), 20.3% did not achieve complete remission (CR) and no factors were significantly associated. The 4-year event-free survival (EFS) rate of the entire cohort was 86%. The 4-year EFS rate was 93% for patients with CR in iPET2 and 52% for patients not achieving CR. The only independent predictor of low EFS was iPET2 response (p < 0.05). Conclusion Our study confirms the prognostic role of PET scan staging and response assessment. Not achieving CR on the iPET2 scan indicates poor prognosis and warrants clinical trial enrollment for a better outcome.

Background: Diffuse large B-cell lymphoma (DLBCL) is considered the commonest subtype of non-Hodgkin lymphoma (NHL) in the world. It is a refractory disease with a high mortality rate due to frequent relapses. Several prognostic parameters are now widely studied for risk stratification and achieving a better outcome. Objectives: In this study, we aim to assess the prognostic value of immunohistochemical expression of CD10, BCL6, and MUM1 independently as surrogate markers for cell of origin (COO) classification of DLBCL and their correlation with clinicopathological characters and survival. Patients and methods: This is a retrospective study conducted on 63 cases of DLBCL, NOS. Full-faced sections were constructed and immunostained for CD10, BCL6, and MUM1. Results: CD10 expression was associated with early-stage (P=0.003), normal serum LDH level (P=0.022), absence of B symptoms (P=0.019), low international prognostic index (IPI) and age-adjusted-IPI (P=0.001) and also associated with longer progression free survival (PFS) (P=0.006). BCL6 expression was associated with centroblastic variant (P=0.005), good ECOG performance status (P=0.038) and low IPI (P=0.004) and also associated with better overall survival (OS) (P=0.028) and PFS (P=0.018). MUM1 expression was associated with advanced-stage (P=0.002) while no significant association was detected with other clinicopathological parameters or survival. Conclusion CD10, BCL6, and MUM1 can be used independently as prognostic immunohistochemical markers for DLBCL that may denote the clinical behavior of the disease and further patients' outcomes.

T-cell prolymphocytic leukemia (T-PLL) is an aggressive post thymic T-cell malignancy which accounts for 2% of mature lymphocytic leukemias in adults. Though typically presenting with a brief history of B symptoms, hepatosplenomegaly, and marked lymphocytosis, erythematous or nodular skin rashes involving the trunk or limbs may be seen in 25-30% of patients, as well as a purpuric rash in a periorbital distribution. Cutaneous involvement typically presents in the context of patients with an established history of T-PLL, but it can less frequently present as an initial symptom heralding the diagnosis. An unusual case of T-PLL is described, presenting initially as palmoplantar ulcerated nodules with an initial biopsy suggestive of perniosis, followed by rapid progression of dark violaceous and bright red papules throughout the body after initiation of Obinutuzumab. The diagnosis of T-PLL was subsequently fully supported by the clinical, laboratory, cytologic, and immunophenotypic findings. This case highlights the importance of a multidisciplinary team approach to address such rare and atypical presentations. This article is protected by copyright. All rights reserved.

Comprehensive studies of primary cutaneous extranodal natural killer/T-cell lymphoma (PCENKTL) are scarce. The objectives of this study are to describe PCENKTL in terms of its clinical features, histopathology, immunophenotypes, and prognosis, and to analyze factors affecting patient survival outcomes. We searched four databases and include studies with extractable data. We also searched the Asan Medical Center database for cases of PCENKTL. We include a total of 289 patients. The mean age at diagnosis was 52.8 years and the female to male ratio was 1:1.2. The most common clinical morphology was a subcutaneous nodule, followed by ulceration. About half of the patients presented with disseminated skin lesions. The median overall survival was 12.0 months and the 5-year survival rate was 22.0%. There was no correlation between the clinical morphology or the histopathological features of the skin lesions with the patient outcomes. Advanced TNM stage, a disseminated skin lesion, tumor location on the leg or trunk, the presence of B symptoms, and a high International Prognostic Index score were associated with a worse prognosis, and chemoradiotherapy was associated with a better survival outcome as compared with chemotherapy alone in univariable analyses. In multivariable analyses, only advanced TNM stage and tumor location on the leg were associated with a worse prognosis. In conclusion, PCENKTL is an aggressive cutaneous lymphoma and its prognosis is associated with TNM stage and tumor location.

Objectives: Hodgkin’s lymphoma (HL) is a B-cell lymphoma characterized by the presence of Reed-Sternberg cells. HL comprises 1% of all cancer cases and 14% of all lymphoma cases. Nodular lymphocyte predominant HL (NLPHL) is a subtype of HL characterized by a unique clinical and histological presentation. The clinical presentation and survival rate of these patients have not been described in Oman. We, therefore, aimed to describe the clinical characteristics and survival rate of patients with NLPHL. Materials and Methods: This is a retrospective cohort study that was conducted in a tertiary care centers in the Sultanate of Oman. All Omani patients with histopathological confirmed diagnosis of NLPHL from 1998 to 2016 were included in this study. The demographic, clinical, and survival rate were recorded and analyzed. Results: Forty-six histologically confirmed cases of NLPHL were analyzed with a median follow-up of 72 months (range: 12–228 months). Median age at diagnosis was 26.5 years (range: 13?53) and 70% of the patients were male. B symptoms were present in 6% and stage I disease was the most common (39%) presenting stage. Patients were treated with radiotherapy (33%), chemotherapy (37%), combined modality (radiotherapy and chemotherapy) (28%), or careful watch-and-wait (2%). Relapses occurred in twelve (26%) patients after a median of 48 months (24–168 months). Two (4%) patients died during followup due to relapse of NLPHL. Conclusion: This is the first study that demonstrates the demographic, clinical, and survival rate of NLPHL patients in Oman. Our study confirms the male predominance in NLPHL patients. The overall 10-year survival was 96%. The outcome was not significantly different with chemotherapy alone or radiotherapy or combined modality. This study showed higher relapse rate; therefore, further study is needed to validate this observation. Keywords: Hodgkin’s lymphoma, NLPHL, Survival, Outcome, Oman.

High-dose chemotherapy and autologous stem-cell transplant (HDC/ASCT) is standard treatment for chemosensitive relapsed classical Hodgkin lymphoma, although outcomes of high-risk relapse (HRR) patients remain suboptimal. We retrospectively analyzed all HRR classical Hodgkin lymphoma patients treated with HDC/ASCT at our institution between 01/01/2005 and 12/31/2019. HRR criteria included primary refractory disease/relapse within 1 year, extranodal extension, B symptoms, requiring more than one salvage line, or positron emission tomography (PET)-positive disease at ASCT. All patients met the same ASCT eligibility criteria. We treated 501 patients with BEAM (n=146), busulphan/melphalan (BuMel) (n=38), gemcitabine( Gem)/BuMel (n=189) and vorinostat/Gem/BuMel (n=128). The Gem/BuMel and vorinostat/Gem/BuMel cohorts had more HRR criteria and more patients with PET-positive disease at ASCT. Treatment with brentuximab vedotin (BV) or anti-PD1 prior to ASCT, PET-negative disease at ASCT, and maintenance BV increased over time. BEAM and BuMel predominated in earlier years (2005-2007), GemBuMel and BEAM in middle years (2008-2015), and vorinostat/GemBuMel and BEAM in later years (2016-2019). The median follow-up is 50 months (range, 6-186). Outcomes improved over time, with 2-year progressionfree survival (PFS)/overall survival (OS) rates of 58%/82% (2005-2007), 59%/83% (2008-2011), 71%/94% (2012-2015) and 86%/99% (2016- 2019) (P

Ziwei Zhou,* Zhe Guo,* Qingqiao Hu, Wei Ding, Chongyang Ding, Lijun Tang Department of Nuclear Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, Jiangsu, People’s Republic of China*These authors contributed equally to this workCorrespondence: Lijun Tang; Chongyang DingDepartment of Nuclear Medicine, The First Affiliated Hospital of Nanjing Medical University, No. 300, Guangzhou Road, Nanjing, 210029, People’s Republic of ChinaTel +14751830090; +15951614327Email tanglijun@njmu.edu.cn; chongyangding@163.comObjective: To explore regional brain glucose metabolic abnormalities of pretreatment stage I/II extranodal natural killer/T-cell lymphoma (ENKTL) patients using positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography (18F-FDG PET/CT) and assess its prognostic value.Methods: Sixty pretreatment stage I/II ENKTL patients were enrolled in this retrospective study and divided into survival (n = 45) and death (n = 15) groups according to their status at the end of follow-up. A control group consisted of 60 healthy subjects. Regional cerebral glucose metabolism was evaluated on a voxel-by-voxel basis using statistical parametric mapping (SPM8) under a certain significance level (P < 0. 001) and voxel threshold (K = 100 voxels).Results: Decreased metabolism was noted in patients, involving the bilateral prefrontal and orbitofrontal cortex, partial parietal and occipital cortex, cingulate gyrus and cerebellum; the sensorimotor cortex was largely spared. Increased metabolism was observed in the bilateral putamen, amygdala, and parahippocampal gyrus. Compared with the survival group, the death group had higher metabolism in the bilateral amygdala, putamen, left thalamus, uncus, and parahippocampal gyrus. Only B symptoms were associated with the increased metabolism of basal ganglia and thalamus (BGT). Patients with high metabolic tumor volume, total lesion glycolysis (TLG) and BGT metabolism had a poor prognosis. TLG and maximum standardized uptake value (SUVmax) LBGT/SUVmaxRight cerebellum were associated with Eastern Cooperative Oncology Group (ECOG) and prognostic index of natural killer lymphoma and Epstein-Barr virus-DNA (PINKE) scores. In multivariate analysis, only ECOG was an independent prognostic factor of both progression-free survival (PFS) and overall survival (OS). PINKE was an independent prognostic factor of OS.Conclusion: Pretreatment stage I/II ENKTL patients exhibited abnormal regional cerebral glucose metabolism. Higher pretreatment glucose metabolism in BGT could predict a relatively poor prognosis but did not surpass the predictive values of ECOG and PINKE in stage I/II ENKTL patients.Keywords: extranodal natural killer/T- cell lymphoma, regional cerebral glucose metabolism, 18F-FDG PET/CT, statistical parametric mapping, prognostic value

Cat scratch disease is caused by the gram-negative intracellular bacterium Bartonella henselae (B. henselae). Human infection occurs mainly through cat scratches and bites. In typical cases, the clinical course is presented by a primary affect on the skin and regional lymphadenitis. The atypical presentation of infection can occur with fever, abdominal, ocular and neurological manifestations. A special feature of the abdominal form is the presence of solitary or multiple hypo/anechoic foci of different size and shape in the liver and spleen. B. henselae infection is one of the most common mimics of malignant lymphoma, when the spleen is involved in combination with B symptoms (weight loss, night sweats and prolonged fever). A history of recent contact with cats and diagnostic IgG titers to B. henselae indicate infection. There is no consensus on the choice of antimicrobial drug and the duration of therapy for systemic forms. The article presents its own experience in the diagnosis and treatment of such patients. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

Hepatosplenic γ δ T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma (PTCL) with aggressive clinical behavior. To date, no standard therapy for HSTCL has been established. This study analyzed the clinical features, treatment, and prognosis for patients with HSTCL to determine the best therapeutic approach. We reviewed the clinical characteristics, treatments, and responses to treatment of patients in our center between January 2001 and June 2021, and also reviewed related literature. Median patient age was 38 years (range 16–60 years) and the patients included eight males and six females. HSTCL in these patients typically presented with B symptoms (71.4%), splenomegaly (100%), and hepatomegaly (50.0%), but lymphadenopathy was extremely rare. In these patients, routine laboratory testing showed elevated lactate dehydrogenase (71.4%), liver dysfunction (42.9%), and decreased fibrinogen (35.7%). In the induction phase, five of the 14 patients received chemotherapy regimens containing anthracycline (CHOP, or CHOP plus bortezomib or Chidamide), and six were treated with non-CHOP chemotherapy. Seven patients responded to induction treatment, four of whom received allogeneic hematopoietic cell transplantation and then achieved a complete response in the consolidation phase. survival time of patients who received alloHCT range from 10 to 27 months. Hepatosplenic γ δ T-cell lacks a standard therapy and is often refractory to conventional chemotherapy regimens. Intensive induction chemotherapy followed by hematopoietic cell transplantation may improve the prognosis of HSTCL.

Diffuse large B-cell lymphoma (DLBCL) comprises approximately 30% of all non-Hodgkin lymphomas. Multiple studies have demonstrated race-based disparities in survival among patients with DLBCL across all stages of disease, in the era both before and after rituximab. The etiology for the racial disparities in survival among patients with DLBCL is still unknown. Moreover, the Revised International Prognostic Index (R-IPI), a tool that predicts the DLBCL patients' outcome, has not yet been validated in African Americans (AA).We conducted a cohort study of patients diagnosed with DLBCL from January 1, 2007, to December 31, 2017, from our tumor registry in a single community-based inner-city cancer center. We abstracted demographic, clinical, histopathologic, treatment, and R-IPI variables. A total of 181 patients (47.5%) with biopsy-proven DLBCL were included in the retrospective analysis. The median age was 65 years, 47% were men, 41% were AA, and 44% were white.The AA group had a younger median age, higher lactate dehydrogenase levels, higher frequency of B symptoms, and higher HIV infection than the non-AA group. The AA group had significantly decreased median overall survival than the non-AA group (15.7 months; 95% confidence interval, 10.3 to 23.9, vs. 93.6 months; 95% confidence interval, 61.5 to 142.6, respectively; P .001). The survival disparities persisted after excluding patients with HIV and who did not receive chemotherapy. In addition, AA race predicts a reduced survival by univariate and multivariate analysis.AA with DLBCL may have a poorer prognosis than the non-AA population. Further studies should investigate the biology of DLBCL in the AA population.

Jiwei Li,1– 3 Jin Li,4,5 Meizuo Zhong,6 Hui Zhou,4,5 Baohua Yu1– 3 1Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, 200032, People’s Republic of China; 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032, People’s Republic of China; 3Institute of Pathology, Fudan University, Shanghai, 200032, People’s Republic of China; 4Department of Lymphoma and Hematology, Hunan Cancer Hospital, Changsha, People’s Republic of China; 5Department of Lymphoma and Hematology, The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, People’s Republic of China; 6Department of Oncology, Xiangya Hospital, Central South University, Changsha, Hunan, People’s Republic of ChinaCorrespondence: Baohua YuDepartment of Pathology, Fudan University Shanghai Cancer Center, Shanghai, People’s Republic of ChinaEmail yubh2014@163.comHui ZhouDepartment of Lymphoma and Hematology, Hunan Cancer Hospital, Changsha, People’s Republic of ChinaEmail zhouhui9403@126.comObjective: Advanced stage extranodal natural killer/T-cell lymphoma (ENKTL) is a distinct type of non-Hodgkin lymphoma and the prognosis of ENKTL is poor with current treatment. This study aimed to investigate the clinical features, treatment strategy and survival outcome in patients with advanced stage ENKTL.Patients and Methods: A total of 107 patients with newly diagnosed advanced stage ENKTL between January 2010 and December 2014 were reviewed from three cancer centers. Survival probability was calculated using Kaplan-Meier and the survival curves were compared by Log rank test. Cox regression analyses was performed to investigate the prognostic factors in ENKTL.Results: The median patient age in our cohort was 42.0 years, with a male to female ratio of around 2.3:1. Over half of the patients had B symptoms (n = 61), high IPI scores (≥ 2, n = 60) and high Prognostic Index of Natural Killer Lymphoma (PINK) scores (≥ 3, n = 69). Elevated LDH level was present in around half of the patients (44/91). Most patients (n = 88) in our cohort originated in upper aerodigestive tract and the remaining 19 cases presented with non-upper aerodigestive tract involvement at first diagnosis. Chemotherapy regimens used in our study mainly include CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) (n = 26), L-asparaginase (L-asp) containing chemotherapy (GELOXD (gemcitabine, l-asparaginase, oxaliplatin and dexamethasone) and SMILE (L-asparaginase, methotrexate, ifosfamide, etoposide, and dexamethasone)) (n = 66). No significant difference between the baseline clinical characteristics was found between the L-asp and CHOP group. The CR rate after treatment was 39.3% (42/107) for the whole cohort. The 3-year progression-free survival (PFS) and 3-year overall survival (OS) rate was 41.0% and 41.5%, respectively. The 3-year PFS (49.2% vs 26.5%, P = 0.048) and 3-year OS (49.4% vs 26.0%, P = 0.030) was significantly higher in the L-asp group than the CHOP group. Patient CR status and PINK score were proved to be significant independent factors affecting OS and PFS by multivariate analysis. The grade 3/4 hematologic toxicity (P = 0.0003) and non-hematologic toxicity (P = 0.0002) occurred more frequently in the SMILE group than the GELOXD group.Conclusion: Our results demonstrated that L-asp containing chemotherapy could provide favorable survival outcomes in patients with advanced stage ENKTL.Keywords: extranodal natural killer NK/T-cell lymphoma, chemotherapy, toxicity

Background Prompt and accurate diagnosis of lymphadenopathy is important, yet there is wide variability in clinical approach and referral patterns, leading to unnecessary investigations and delays in diagnosis. To address this, a lymph node diagnostic clinic (LNDC) was established at our tertiary referral center. Patients and Methods We retrospectively analyzed data from 320 consecutive patients referred to the LNDC from March 2008 to March 2020, to describe their management and outcomes. Results The most common diagnoses were reactive (57%) and malignant lymphadenopathy (28%). In those with reactive lymphadenopathy, 33% did not undergo further investigations, 37% underwent imaging only, and 29% underwent biopsy. For malignant lymphadenopathy, diagnosis was made at a median (interquartile range) of 9 (6-16) days from first LNDC review, with the decision to biopsy made at the first LNDC review in 95% of cases. Clinical features significantly associated with malignancy included age > 45, B symptoms, history of malignancy, and lymphadenopathy that was ≥ 2 cm, in multiple regions, bilateral, multiple nodes, or supraclavicular. At least 3 of these features were present in 88% of patients with malignant lymphadenopathy. Ultrasound had a sensitivity of 98% and negative predictive value of 97% for detecting malignant lymphadenopathy. Conclusion A dedicated LNDC in a tertiary referral center facilitates rapid assessment and diagnosis of lymphadenopathy through a risk-stratified model of management. Ultrasonography, as well as the presence of defined clinical risk factors, were most useful to differentiate benign from malignant lymphadenopathy.

Background The purpose of this study was to examine the factors associated with disparities in overall survival (OS) by race in pediatric diffuse large B‐cell lymphoma (DLBCL) patients. Methods We evaluated clinical features and survival among patients ≤21 years of age diagnosed with stage I–IV DLBCL from 2004 to 2014 from the National Cancer Database (NCDB) using a multivariable Cox proportional hazards model. Results Among 1386 pediatric patients with DLBCL, 1023 patients met eligibility criteria. In unadjusted analysis, Black patients had a significantly higher overall death rate than White patients (HRBlack vs. White 1.51; 95% CI: 1.02–2.23, p = 0.041). The survival disparity did not remain significant in adjusted analysis, though controlling for covariates had little effect on the magnitude of the disparity (HR 1.46; 95% CI 0.93–2.31, p = 0.103). In adjusted models, presence of B symptoms, receipt of chemotherapy, stage of disease, and Other insurance were significantly associated with OS. Specifically, patients with B symptoms and those with Other insurance were more likely to die than those without B symptoms or private insurance, respectively (HR 1.75; 95% CI 1.22–2.50, p = 0.002) and (HR 2.56; 95% CI, 1.39–4.73, p = 0.0027), patients who did not receive chemotherapy were three times more likely to die than those who received chemotherapy (HR 3.10; CI 1.80–5.35, p, The purpose of this study was to examine the factors associated with disparities in overall survival (OS) by race in pediatric diffuse large B‐cell lymphoma (DLBCL) patients. Black patients had a significantly higher overall death rate than White patients (HRBlack vs. White 1.54; 95% CI: 1.01–2.35, p =0.044). Our results suggest that racial disparities in survival may be mediated by clinical and treatment parameters.

We aimed to evaluate the prognostic value of 18F-FDG PET/CT in patients with relapsed or refractory T-Lymphoblastic lymphoma (T-LBL) undergoing hematopoietic stem cell transplantation (HSCT). PET/CT was performed in 21 consecutive relapsed or refractory T-LBL patients scheduled for HSCT. All PET/CT images were assessed using the Deauville criteria, and patients were divided into negative (Deauville ≤ 3) and positive (Deauville > 3) groups for comparison. The predictive value of sex, age, Ann Arbor stage, presence of B symptoms, lactate dehydrogenase level, presence of extranodal disease, and PET/CT results before and after HSCT were evaluated. Kaplan–Meier analysis showed that only PET/CT after HSCT (post-PET) was correlated with progression-free survival (PFS) (P = 0.030). The Cox regression model also showed that the post-PET-positive group had a higher hazard ratio (HR) than the negative group (HR = 3.884 and P = 0.049). However, none of the evaluated factors were predictive of overall survival (OS). Pre-PET cannot predict the PFS and OS of patients with T-LBL undergoing HSCT, which means that 18F-FDG PET/CT cannot be used for identifying patients who can benefit from HSCT. Post-PET is not predictive for OS in patients with T-LBL undergoing HSCT. However, post-PET showed strong correlations with PFS, which means that it may be useful for guiding subsequent clinical treatment decisions.

Gastric diffuse large B cell lymphoma (DLBCL) represents the majority of all gastric lymphomas. We report a series of gastric DLBCL diagnosed and treated in a single center, between 2010 and 2018 (included). We retrospectively analyzed the population demographic features, treatment outcomes and survival. One-hundred-and-one patients were studied, 50.5% males and median age of 64 years [23–94]. Lugano staging was I in 16.8%, II1 in 20.8%, II2 in 10.9%, IIE in 13.9% and IV in 34.7% of cases. Twenty percent had Helicobacter pylori infection. R-CHOP-like therapy was used as first line in 96.9% of the patients. A complete response was achieved in 80% after first line therapy. At 3-years of follow-up (FU), 54% were in complete remission. The mean FU time was 73.6 months. Median overall survival and median progression free survival were not reached. We identified seven factors with negative impact in survival: age above 65 years-old (p

Human immunodeficiency virus (HIV) infection is associated with an increased risk of aggressive lymphoma, especially diffuse large B cell lymphoma (DLBCL). There are few data regarding HIV-associated DLBCL in China. Therefore, we analyzed the characteristics and outcomes of patients with HIV-associated DLBCL from our center. We retrospectively studied HIV-infected patients with DLBCL from 2011 to 2019. Data on HIV infection and lymphoma characteristics, treatments and outcomes were retrieved and analyzed. In 78 patients with HIV-associated DLBCL, most had poor performance status (PS) (74%), elevated lactate dehydrogenase (LDH) levels (95%), B symptoms (74%), advanced Ann Arbor stages (81%), bulky diseases (64%) and extranodal involvement (70%) at diagnosis. The median CD4+ T cell count was 162/µl, and 26 patients were already on combination antiretroviral therapy (cART) treatment at diagnosis of DLBCL. Elevated whole blood EBV DNA copy number was detected in 38 patients (66%, 38/58). Of the 45 patients evaluated at the end of treatment, 26 (58%) achieved CR, 6 (13%) achieved PR and 6 (13%) experienced progressive disease. The 2-year progression-free survival (PFS) and overall survival (OS) rates were 56.4% and 62.7%, respectively. Factors associated with decreased PFS and OS in univariate analysis were unfavorable PS and high international prognostic index. Elevated EBV DNA copy number was inclined to be associated with worse outcome. We did not observe a significant difference in survival between R-EPOCH and R-CHOP regimens. In our population, patients with HIV-associated DLBCL presented with aggressive characteristics and exhibited poor survival outcomes, even in the modern cART era.

Waldenstrom’s macroglobulinemia (WM) is an indolent B-cell non-Hodgkin lymphoma characterized by lymphoplasmacytic histology in the bone marrow with monoclonal IgM. Median survival can be in excess of 10 years. The 5-year cumulative incidence of death is low at about 10%. One-third of all-cause specific mortality is due to the lymphoma for which histologic transformation (HT) is rare. Here we present a case of a 60-year-old man with longstanding untreated WM, presenting with minimally symptomatic transformation to diffuse large B-cell lymphoma (DLBCL), with an accompanying review of the literature. Transformed WM, diagnosed greater than 5 years, has a reported survival period of 8 - 9 months. This case highlights that after a decade of continued stability in WM, not requiring treatment, an acute change in laboratory data with minimally progressive IgM levels, in the absence of B symptoms and clinical findings, may be the harbinger of transformation and at the time of diagnosis can have a rapidly deteriorating clinical course. In this case, the tripling of the lactate dehydrogenase (LDH) as the primary drastic change demonstrates the importance of the rapid increase in LDH as a singly reliable marker for HT. Late transformation has been borne out as a negative variable as the generally indolent course of WM is curtailed with the poor outcome in HT. Although MYD88 wildtype is a possible predictive factor for transformation, it is unclear if late transformation is clonally or non-clonally related and further molecular investigation is needed.

Background: Adult Onset Stills Disease (AOSD) and Kikuchi Fujimoto Lymphadenitis (KFL) are both rare Rheumatological diseases. Co-occurrence is very rare. Case Presentation: This is a case of AOSD and KFL co-occurrence presenting with polyarthritis, rash, and B Symptoms. Elevated serum lactate dehydrogenase and ferritin with anemia and lymphadenopathy raised suspicions of lymphoma. Histology confirmed KFL and clinical features, and raised ferritin confirmed AOSD. After starting sulfasalazine, the patient was admitted to the hospital with ‘Drug Reaction with Eosinophilia and Systemic Symptoms’ (DRESS) Syndrome. Prior to sepsis exclusion, Gentamicin was given and subsequent hearing loss was noted. Sustained remission was achieved with Tocilizumab and azathioprine. Tocilizumab was stopped during pregnancy. Conclusion: The diagnostic challenges of two rare overlapping Rheumatological diseases are highlighted. An early distinction of disease flares or drug reactions from sepsis is difficult. Risk-benefit ratio consideration of continuing successful treatments in pregnancy is acknowledged.

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive lymphoma that accounts for approximately 18% of natural killer/T-cell lymphomas.1 AITL typically affects older men and manifests at nodal and extranodal sites with B symptoms, lymphadenopathy, hematologic abnormalities, and, in up to 50% of cases, cutaneous manifestations.1, 2, 3 We report a case of AITL with a severe skin eruption developing during checkpoint inhibitor therapy.

Introduction: Integrated PET/CT has been shown to be more accurate for lesion localization and characterization in lymphoma than PET and CT alone. Addition of CECT to PET/CT changed management of lymphoma in only about 10% of patients, while FDG/PET resulted in a management change in almost 50 % of HL patients compared with CECT alone. The majority of patients with high-grade non-Hodgkin’s lymphomas (NHL) are cured with combination treatments. However, acute and long-term toxicities impair survival. Patients with poor-risk, progressive, or relapsed disease; the goal is to improve survival with a strategy using more intensive therapy. Hence, the focus of management has now shifted towards reducing the treatment toxicity and long-term side effects while maintaining favorable outcomes especially in low-risk patients. The successful application of this tailored approach is dependent on an accurate and non-invasive diagnostic test that would reflect the true extent of disease as well as its viability early during the course of treatment. Metabolic parameters in malignant lymphoma: An imaging task force was created to update the relevance of existing imaging for staging, assessing bulk, and bone marrow involvement (BMI); the role of interim PET; standardization of PET reporting; and role for quantitative evaluation using PET and CT. A clinical task force assessed the current relevance of Ann Arbor and how best to incorporate PET/CT into staging lymphoma, the relevance of B symptoms and bone marrow biopsy (BMB), as well as to create recommendations relevant to both FDG-avid and non-avid lymphomas. The revised IWG response criteria (rIWG) incorporated FDG PET to accurately assess end-therapy persistent masses in both NHL and HL. PET/CT in surveillance assessment: Surveillance CT or PET-CT scans are widely used because of the impression that treatment at relapse is more likely to be effective when the disease is in a preclinical stage with a small tumor burden: the earlier the detection of disease the higher the treatment efficacy. The authors concluded that the routine use of surveillance PET in HL patients entering complete remission after first-line treatment should be reserved for high-risk patients. Current guidelines warrant that, once the first clinical remission is obtained, surveillance should be grounded on frequent physical examination and routine blood tests. By contrast, a significant proportion of these patients undergo follow-up CT radiological studies and fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT, especially in the first 2 years after therapy. FDG-PET/CT follow up exams may be associated with false positive results. These confounding findings add up to the already significant FDG-PET/CT expense, which in turn is significantly more resource-consuming when compared to clinical and biochemical evaluation. CONCLUSION: PET/CT imaging for surveillance in malignant lymphoma may be useful in special subtypes of both Hodgkin and non-Hodgkin lymphoma.

Peripheral T cell lymphomas are rare malignancies with aggressive course, with several different subtype described in the 2016 WHO classification. Their distribution across the world is heterogenous, with marked difference between Western and Asian country. Their clinical presentation often comprise extra-nodal involvement, B symptoms and immune system disorder which can lead to wrong diagnosis orientation. Make a right diagnosis need a experienced pathologist in close collaboration with clinical datas. Peripheral T cell lymphomas are in general associated with poor prognosis when treated with anthracyclines-based regimen, and several studies and trials focused on the use of intensified regimen or novel targeted agents, whose proper indication still remain to be clarified.

In humans B‐symptoms refer to systemic symptoms of lymphoma such as fever, weight loss, and night sweats and influence the prognosis of patients. In canine lymphoma, substage B is used to describe any clinical sign observed. Aim of the retrospective study was to compare the prognostic value of substage B with B‐symptoms to predict treatment response and survival in canine nodal diffuse large B‐cell lymphoma. Affected dogs treated with CHOP chemotherapy between 2008 and 2019 were included. B‐symptoms were defined by weight loss greater than 10% of normal weight, fever and the occurrence of unexplained resting tachypnoea substituted human night sweats. Substage B was defined as any symptoms but lymphadenopathy. Fifty‐five cases were included. B‐symptoms were present in 20/55 (36%) and substage B in 40/55 (74%) patients. No significant associations between B‐symptoms or substage B and weight, sex, breed, WHO stage and lymphoma grade were found. Treatment response was negatively associated with both substage B (P = .02) and B‐symptoms (P = .001). B‐symptoms significantly decreased progression free survival (PFS) (95 vs 330 days, P = .001) and lymphoma specific survival (LSS) (160 vs 462 days, P = .001). Data showed that B‐symptoms might be a more reliable prognostic indicator than substage B in canine nodal diffuse large B‐cell lymphoma. Prospective studies assessing B‐symptoms in a larger cohort of patients and in other common lymphoma types are warranted. The abstract was presented at the fourth meeting of the European Canine Lymphoma Network Group in Lugano, 22 June 2019 and published in the proceeding of the meeting on the page 26.

There are limited data regarding the combined value of the pretransplant Deauville score (DS) from a positron emission tomography scan and clinical risk factors in patients with relapsed/refractory aggressive non-Hodgkin lymphoma (NHL). We performed a retrospective analysis to assess the prognostic role of pretransplant DS in patients with relapsed/refractory aggressive NHL who underwent salvage chemotherapy and autologous stem cell transplantation (ASCT). We identified 174 eligible patients between January 2013 and March 2019. In multivariable analysis, pretransplant DS, B symptoms, and secondary International Prognostic Index (sIPI) were independent risk factors for event-free survival (EFS). These variables were used to derive an integrated risk score that categorized 166 patients with available information for all risk factors into 3 groups: low (n = 92; 55.4%), intermediate (n = 48; 28.9%), and high (n = 26; 15.7%). The new prognostic index showed a strong association with EFS (low-risk vs intermediate-risk hazard ratio [HR], 3.94; 95% confidence interval [CI], 2.16-7.17; P < .001; low-risk vs high-risk HR, 10.83; 95% CI, 5.81-20.19; P < .001) and outperformed models based on clinical risk factors or DS alone. These results were validated in 60 patients from an independent external cohort (low-risk vs intermediate-risk HR, 4.04; 95% CI, 1.51-10.82; P = .005; low-risk vs high-risk HR, 10.49; 95% CI, 4.11-26.73; P < .001). We propose and validate a new prognostic index that risk-stratifies patients undergoing salvage chemotherapy followed by ASCT, thereby identifying patients at high risk for posttransplant treatment failure.

INTRODUCTION: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell neoplasm caused by the Human T-cell Leukemia Virus Type 1 (HTLV-1). HTLV-1 infects up to 10 million people worldwide and is most endemic in Southwestern Japan, Western Africa, the Caribbean basin and South America. Cutaneous signs of ATLL are varied and may consist of macules (M), plaques (P), multiple papules (MP), tumoral nodules (TN), erythroderma (E) or mixed-lesions (≥2 predominant lesions, ML). M and P forms are believed to carry a better prognosis. However, data on cutaneous presentation of ATLL remains scarce. Herein, we report cases of ATLL with cutaneous involvement diagnosed in 4 Latin American countries over the last 3 decades. METHODS: We retrospectively analyzed patients (pts) diagnosed with ATLL between January 1995 and December 2019. ATLL subtypes were classified according to the Shimoyama criteria into acute, lymphomatous, chronic and smoldering. Primary cutaneous tumoral (PCT) variant was classified according to the 2019 International Revised ATLL Consensus. We designed 2 cohorts: the first, ATLL pts with cutaneous involvement, and the second, matched cases without cutaneous involvement. We determined the type of skin lesion as well as the survival associated with the various types of skin lesions. We compared the frequency of clinical features using Fisher's exact test. Treatment response was assessed according to Tsukasaki et al. (JCO 2009) criteria. To be classified as complete response (CR), partial response, and stable disease, these had to persist for a period of at least 4 weeks. We analyzed survival data according to ATLL subtype, cutaneous involvement status, and type of skin lesion using the Kaplan-Meier method and Log rank test. RESULTS: A total of 169 pts with ATLL were identified; 63 had cutaneous involvement and 106 did not. Clinical features are shown in Table 1. In both groups the median age was 57 years with a female predominance. Cutaneous involvement was most frequently found in acute (41%) and lymphomatous (37%) ATLL pts. The E (24%) and P (22 %) types were the most frequent skin lesions. Disease stage, presence of B symptoms, hypercalcemia, ECOG ≥2, elevated LDH, and IPI/ PIT score were not different among groups. Table 2 and Table 3 summarize the first-line therapy used and response rates. The use of first-line zidovudine plus interferon alpha (AZT-IFN), regardless of the type of skin lesion, resulted in relatively high response rates [overall response (OR) 100%, n=8; CR 62.5%] as compared to multi agent-chemotherapy (OR 33.3%, n=12). Overall, the presence of cutaneous involvement was associated with better overall survival (OS) compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], p CONCLUSION: In Latin American pts with aggressive ATLL, cutaneous involvement appears to be associated with better survival compared to non-cutaneous involvement. PCT subtype, an ATLL variant characterized by isolated skin lesions with no organ involvement and poor outcome, appeared to have a better prognosis compared to acute and lymphomatous ATLL forms. P and MP skin lesions were both associated with better survival. Hypercalcemia was found as an independent prognostic factor for survival in pts with cutaneous involvement. Finally, AZT-IFN appears to be reasonable first-line option for aggressive ATLL subtypes with cutaneous involvement regardless of the type of skin lesion at diagnosis, based on the relatively high response rates observed in this subset; further investigation in randomized clinical trials is needed. Disclosures Peña: Janssen: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Sandoz: Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees; Amgen: Speakers Bureau; BindingSite: Research Funding. Idrobo:Takeda: Honoraria, Speakers Bureau; Tecnofarma: Honoraria, Speakers Bureau; Abbvie: Honoraria, Speakers Bureau; Janssen: Honoraria, Speakers Bureau; Amgen: Honoraria, Speakers Bureau. Altamirano:Hospital Nacional Guillermo Almenara Irigoyen: Other: Servicio de Hematologia. Perini:Abbvie: Speakers Bureau; Janssen: Honoraria, Speakers Bureau; Takeda: Honoraria. Castillo:Pharmacyclics: Consultancy, Research Funding; Beigene: Consultancy, Research Funding; Abbvie: Research Funding; Kymera: Consultancy; TG Therapeutics: Research Funding; Janssen: Consultancy, Research Funding. Ramos:NIH: Research Funding. Villela:amgen: Speakers Bureau; Roche: Other: advisory board, Speakers Bureau.

Background: The prognosis of Non-Hodgkin Lymphoma (NHL) depends on the type of lymphoma, the extension of the disease and the response to therapy. Aim: To evaluate the prognostic value of pretreatment and interim PET/CT compared to classic prognosis factors and body composition measurement (sarcopenia, adipopenia) in patients with recently diagnosed NHL. Material and Methods: Patients with recently diagnosed NHL who had staging 18F-FDG PET/CT performed between December 2008 and August 2018 were selected. Age, gender, weight, height, B symptoms, laboratory tests, pathology, staging PET/CT findings (Ann Arbor, number of nodal groups and extranodal sites involved, Bulky, maximum standardized uptake value (SUVmax), metabolic tumor volume (MTV), and total lesion glycolysis), Computed Tomography findings (psoas muscle mass index, psoas area, psoas density, subcutaneous fat index (all of them at L3 level), and Deauville score (Lugano Criteria) were recorded. The prognostic value of each of these factors was assessed using Cox multivariable regressions. Results: Of 138 NHL studied patients (median 61 y, 15-87 y, 60.4% men), 31 of them died due to the disease. The median follow-up was 39 months (1-115 months). The strongest prognostic factors were: B symptoms (p < 0.01), anemia (p < 0.01), hypoalbuminemia (p: 0.01), sarcopenia (p < 0.01), adipopenia (p < 0.01), number of node groups involved (p < 0.01), MTV (p < 0.01), and a bad response in interim PET/CT (p < 0.01). In a comparative Cox multivariable analysis, interim PET/CT was the independent variable with the highest significance (p < 0.01). Conclusions: Early treatment response assessed by interim PET/CT is the strongest prognostic factor in NHL patients.

A 10-year-old boy with autoimmune hemolytic anemia (AIHA) was treated at a local setup with repeated blood transfusions and steroids considering an idiopathic etiology. After 7 months of unresponsiveness, the child started developing recurrent fever with night sweats and pain in the abdomen which was diagnosed as tuberculosis on account of granulomatous necrotizing lesion on computed tomography (CT)-guided lymph node biopsy and treated for the same. Four months later, when the child presented to our center in anemic heart failure, work-up with positron emission tomography-CT and excisional biopsy of mesenteric lymph node led to the diagnosis of advanced Hodgkin's lymphoma with B symptoms. AIHA in pediatric age group, unlike in adults, is usually secondary. Our report stresses on a thorough evaluation of AIHA as it can mask a malignancy for a long duration and alter its course if treated with steroids, making the patient resistant to further chemotherapy.

Double-hit/triple-hit lymphomas (DH/THLs) are high-grade B-cell lymphomas with MYC and BCL2 rearrangements and/or BCL6 rearrangements, which have poor outcomes after standard chemoimmunotherapy. This retrospective study analyzed 51 patients (range, 19 to 82 y) diagnosed from 2016 to 2019 and treated for DH/THL (n=34 MYC/BCL6 DHL, n=14 MYC/BCL2 DHL, n=3 THL) at one institution in South China. Extranodal lesions occurred in 32 patients (62.7%), more frequently in MYC/BCL6 DHL (22/34, 64.7%) than in MYC/BCL2 DHL (7/14, 50%). The most common extranodal sites were the stomach (8/32, 25.0%) and intestine (5/32, 15.6%). Most patients (33/45, 73.3%) presented with Ann Arbor stage III/IV. Interestingly, 14.3% (4/28) of MYC/BCL6 DHL tumors showed diffuse, medium-intensity CD30 expression. Epstein-Barr virus-encoded RNA was positive in 3 cases, all MYC/BCL6 DHL. Among 48 patients (94.1%) with follow-up data, 18 (37.5%) died owing to the disease, and the median survival was 5.5 months. Germinal center B cells were observed more frequently in MYC/BCL2 DHL (14/14, 100.0%) than in MYC/BCL6 DHL (15/34, 44.1%; P

Primary bone lymphoma is a rare presentation of non-Hodgkin lymphoma. It accounts for a maximum of 1–2% of all non-Hodgkin lymphomas in adults. Primary bone lymphoma is diagnosed in focal lesions of one or more bones; soft tissue and regional lymph nodes may be involved too. The exclusion criteria are only bone marrow damage and involvement of distant lymph nodes. The first symptoms include intractable bone pain often accompanied by local edema, the formation of a tumor mass in the affected area; B symptoms occasionally join. Local lesions of long tubular bones in the diaphysis and metadiaphysis regions are more common (80%), while multifocal lesions are less frequent (20%). Diagnosis of lesions of the bone tissue in its primary and secondary involvement is based on the use of all available research methods (radiography; computed, magnetic resonance and positron emission tomography). Differential diagnosis requires an immunohistochemical study with determination of the expression of total leukocyte antigen, B-cell and T-cell markers, and clonality in one of immunoglobulin light chains κ or λ, bcl 2 and bcl 6, ALK, proliferative activity of Ki-67. Evaluation of the effectiveness of various treatments for primary bone lymphoma is complicated by a small number of observations and the absence of a uniform treatment strategy. CHOP-like chemotherapy cycles are often used as first-line therapy. Personalized therapy involves immunochemotherapy, radiation therapy and surgical treatment — endoprosthetics.

Primary Lymphoma of bone (PBL) is an uncommon extranodal tumor accounting for 1% of all malignant lymphomas. The incidence of PBL is so rare that many of its aspects remain unknown. We retrospectively analysed our data in order to know clinical characteristics and treatment outcome in Indian population in chemo-immunotherapy era. We identified 49 patients [2007–2019] (median age 52 years) of which, 35 (71.4%) were males. Nearly one-third patients (n = 18; 36.8%) were elderly (Age > 60). The most common histological subtype was DLBCL. Local pain /swelling (n = 23; 47%) and B symptoms (n = 20; 44.4%) was the most common presentation. Spine was the most frequently involved site (n = 25; 51%) followed by pelvis (n = 17; 34.7%). One third patients had poor ECOG-PS ≥ 2, (n = 16; 32.6). More than 50% of the population presented with IPI score ≥ 2 (n = 25; 55.5%). Majority of the patients presented with Ann-Arbor stage IV disease (n = 31; 63.2%). (n = 32; 71.1%) cases received chemotherapy alone and (n = 13; 28.9%) patients were treated in combination with local radiotherapy. R-CHOP was the most common treatment regimen given to patients (n = 43; 95.5%). Overall, three-fourth patients (n = 36; 80%) achieved a complete response. At a median follow-up of 45 ± 2 (range 3–144) months, 4-year OS (Overall Survival) and PFS (Progression free survival) was 83.1% and 74.5%, respectively, using Kaplan–Meier survival curves. Prognostic factors for OS on multivariate analysis were ECOG-PS 0–1 [p = 0.05], age

Rationale and Objectives Radiomic analysis of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) images enables the extraction of quantitative information of intratumour heterogeneity. This study investigated whether the baseline 18F-FDG PET/CT radiomics can predict treatment response and survival outcomes in patients with Hodgkin lymphoma (HL). Materials and Methods Thirty-five patients diagnosed with HL who underwent 18F-FDG PET/CT scans before and during chemotherapy were retrospectively enrolled in this investigation. For each patient, we extracted 709 radiomic features from pretreatment PET/CT images. Clinical variables (age, gender, B symptoms, bulky tumor, and disease stage) and radiomic signatures (intensity, texture, and wavelet) were analyzed according to response to therapy, progression-free survival (PFS), and overall survival (OS). Receiver operating characteristic curve, logistic regression, and Cox proportional hazards model were used to examine potential predictive and prognostic factors. Results High-intensity run emphasis (HIR) of PET and run-length nonuniformity (RLNU) of CT extracted from gray-level run-length matrix (GLRM) in high-frequency wavelets were independent predictive factors for the treatment response (odds ratio [OR] = 36.4, p = 0.014; OR = 30.4, p = 0.020). Intensity nonuniformity (INU) of PET and wavelet short run emphasis (SRE) of CT from GLRM and Ann Arbor stage were independently related to PFS (hazard ratio [HR] = 9.29, p = 0.023; HR = 18.40, p = 0.012; HR = 7.46, p = 0.049). Zone-size nonuniformity (ZSNU) of PET from gray-level size zone matrix (GLSZM) was independently associated with OS (HR = 41.02, p = 0.001). Based on these factors, a prognostic stratification model was devised for the risk stratification of patients. The proposed model allowed the identification of four risk groups for PFS and OS (p Conclusion HIR_GLRMPET and RLNU_GLRMCT in high-frequency wavelets serve as independent predictive factors for treatment response. ZSNU_GLSZMPET, INU_GLRMPET, and wavelet SRE_GLRMCT serve as independent prognostic factors for survival outcomes. The present study proposes a prognostic stratification model that may be clinically beneficial in guiding risk-adapted treatment strategies for patients with HL.

Background: Epstein-Barr virus (EBV) is a ubiquitous virus belonging to γ-Herpesvirus subfamily, infecting B cells, T cells, Natural killer (NK) cells & causes both benign and malignant diseases. It has been detected in large subset of Hodgkin lymphoma (HL) cases around the world, especially in countries with poor socioeconomic conditions and among younger age. Limited studies are available reflecting the Indian scenario of HL and EBV association. EBV positivity in Indian HL varies from 28-97% Majority of these studies employed Immunohistochemistry (IHC) for LMP1, a few performed In Situ Hybridisation (ISH) for EBER. Objective: To study the association of EBV in classical HL by immunohistochemical expression of latent membrane protein 1 (LMP1) antigen in North Indian population and to correlate it with different demographic variables & subtypes of HL. Materials and Methods: Observational study including 26 untreated HL cases diagnosed on lymph node excision biopsy. IHC was performed for EBV LMP1, CD15, CD30, CD45, CD3, CD20. Results: Patients ranged in age from 5-55years (median 18yrs), with M:F ratio of 3.3:1. Palpable lymphadenopathy was found in all cases followed by pallor (64%), B symptoms (50%), nodal pain (30.8%) & bulky disease (19.2%). Maximum number of patients were in Stage I (65.4%) followed by stage II&III (15.4% each) & stage IV (3.8%). Mixed cellularity HL comprised 77%, lymphocyte depleted 11.5%, nodular sclerosis 7.7% & lymphocyte rich 3.8%. IHC for EBV LMP1 was positive in 73.1% cases. Mixed cellularity HL showed an association in 70% cases. Conclusions: HL in India is a disease of young males, with mixed cellularity as the commonest subtype, highly associated with EBV and presentation at an early stage.

Background The programmed death receptor (PD-1) and ligand (PD-L1) pathway act by suppressing the antitumor response in chronic Hodgkin lymphoma (cHL). In this study, we aimed to investigate the effect of PD-1, PD-L1, and Epstein-Barr virus (EBV) positivity on prognosis at the initial diagnosis of cHL. Material and Methods Thirty-six patients with cHL were retrospectively analyzed. PD-L1 staining was performed for RS cells and tumor microenvironment in the biopsy materials of cases. The presence of EBV was investigated by EBER (EBV-encoded RNA) method in tumor cell. P Results The presence of advanced-stage disease, B symptoms, intermediate or high-risk international prognostic index (IPS), and extranodal involvement were found to be related to both PD-L1 positivity and EBV positivity in RS cells. PD-L1 positivity in RS cells was also associated with EBV positivity. There were 6 (16.7%) triple-positive (EBV+, RS-PD-L1+, mic-PD-1+) patients. All of these patients had advanced-stage disease, B symptoms at the time of diagnosis, and intermediate-high IPS score, and 4 of 6 patients had extranodal involvement. This group also had significantly shortened overall survival compared with others (38.4 months vs. 67.9 months P = .024). Conclusion Our data suggest that there is correlation between PD-L1 positivity and EBV positivity in tumor RS cells that are also associated with extranodal involvement, intermediate and high IPS score, presence of B symptoms, and advanced-stage disease. In addition, we identified a group of triple-positive (EBV+, RS-PD-L1+, mic-PD-1+) cHL patients who have a very high-risk disease.

OBJECTIVE To describe clinical features and treatment options in pediatric patients with ALCL (Anaplastic large cell lymphoma) and their outcome over a span of 10 years. STUDY DESIGN A retrospective-observational study. PLACE AND DURATION OF STUDY Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from January 2005 to December 2015. METHODOLOGY Medical records of pediatric patients with anaplastic large cell lymphoma was retrospectively collected after IRB approval. Data was reviewed for patients confirmed on histopathology and age less than 20 years at the time of diagnosis to see clinical features and treatment outcomes. Descriptive statistics were applied. RESULTS A total of 40 children, 27 males (67.5%) and 13 females (32.5%) with ALCL (CD30 +), were reviewed. B symptoms were present in 32 (80%) patients, nodal involvement in 39 (97.5%), and mediastinum involvement was present in 8 (20%) patients. Visceral (lung, liver, spleen) and cutaneous involvement was seen in 16 (40%) and 6 (15%) patients, respectively. ALK was positive in 19 patients (48%) and Bone marrow was involved in 3 patients (7.5%). Stage III was seen in 29 (72.5%). All patients were treated on ALCL 99 protocol. Five-year EFS (event-free survival) and OS (overall survival) was 30 and 60%, respectively. There were 7 relapses, 2 progressive disease, 16 death and 3 refusal for treatment. CONCLUSION This analysis shows poor outcomes in pediatric ALCL. The most common cause of mortality was hematological toxicity and febrile neutropenia associated with it. Supportive care needs to be improved. Key Words: Clinical features, Outcomes, Anaplastic large cell lymphoma (ALCL), Children.

Background The CHOP regimen comprising cyclophosphamide, doxorubicin, vincristine, and prednisone is a basic chemotherapeutic regimen for diffuse large B cell lymphoma (DLBCL). Addition of rituximab (R) to chemotherapy has led to better efficacy than other regimens in clinical trials. However, data of clinical characteristics and outcomes of patients with DLBCL are scarce. Therefore, this study reports the clinical characteristics, treatment, and outcomes of patients with DLBCL in our hospital. Methods We conducted a retrospective analysis of newly diagnosed DLBCL patients treated with CHOP-like or R-CHOP-like regimens at our hospital between 2011 and 2018. We analyzed the data on demography, clinical characteristics, treatment, treatment response, and survival time. Both univariate and multivariate analyses were applied. Results In total, 570 newly diagnosed DLBCL patients were included, of which 133 were treated with CHOP-like regimens and 437 with R-CHOP-like regimens. The overall response rate was 83.3%. Germinal center B-cell-like (GCB) subtype, R-CHOP-like treatment, Ann Arbor stage I-II, not more than 1 extranodal disease site, Eastern Cooperative Oncology Group (ECOG) score ≤1, normal serum lactate dehydrogenase (LDH) level, normal serum β2 microglobulin (β2-MG) level, absence of B symptoms, and lower International Prognostic Index (IPI) or National Comprehensive Cancer Network-International Prognostic Index (NCCN-IPI) scores were associated with longer overall survival (OS) and progressionfree survival (PFS), and were favorable prognostic factors for OS and PFS. Only GCB subtype, R-CHOPlike treatment, absence of B symptoms, and lower IPI or NCCN-IPI scores were independent favorable prognostic factors for OS and/or PFS. Neither IPI nor NCCN-IPI could accurately and precisely predict the prognosis of high-risk DLBCL patients. Conclusions This analysis of newly diagnosed DLBCL patients indicates that patients treated with R-CHOP-like regimens or with GCB subtype exhibited better outcomes. Further, IPI and NCCN-IPI have limited prognostic values in high-risk DLBCL patients.