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Refractory/relapsed idiopathic multicentric Castleman disease (R/R iMCD) has limited treatment options. With studies showing increased mTOR activation in iMCD patients, sirolimus becomes an attractive and promising therapy for R/R iMCD. Here we report the results of a retrospective study involving 26 R/R iMCD patients treated with sirolimus-containing regimen. The median age at sirolimus initiation was 40.5 years (23–60), with a median prior treatment line of 2 (1–5). 18 patients (69.2%) achieved symptomatic and biochemical response, with a median time to at least overall partial remission of 1.9 months (0.5–14.6). The median follow-up time from sirolimus initiation was 11.7 months (1.6–50.7) and the median time to next treatment (TTNT) was 46.2 months. No patients died at the end of follow-up. Most of the patients in the cohort are in ongoing responses and continue sirolimus therapy. Sirolimus is well tolerated with minor adverse effects. In conclusion, sirolimus is effective for R/R iMCD patients with good tolerance. [ABSTRACT FROM AUTHOR]

Background: Castleman disease (CD), also known as angiofollicular lymph node hyperplasia or large lymph node hyperplasia, is a rare medical condition. Despite its rarity, it exhibits diverse clinical presentations and outcomes, which pose challenges for comprehensive understanding and management. This study aims to shed light on the demographics, associations, and outcomes of CD by conducting a retrospective analysis. Methods: The National Inpatient Sample (US) was used to identify patients with the diagnosis of Castleman disease using ICD-10 diagnosis code D47.Z2, during the years 2016–2019. Data was collected on demographics, associated diagnoses, treatments and outcomes. Data analysis was performed using STATA Version 17, College Station, TX: Stata Corp LLC. Results: Our study identified 791 hospitalizations involving adult CD patients. The mean age of these patients was 52.4 years, with a male predominance (56.1%). Whites comprised the largest racial group affected (50.1%). Most patients were covered by Medicare (39.6%). The majority received treatment in urban teaching hospitals (84.0%) and large-bed size facilities (62.5%). In-hospital mortality was low at 2.8%, with an average length of stay of 7.5 days and average total charges of $109,308. Common associations included acute kidney injury (27.0%), congestive heart failure (17.1%), sepsis (16.4%), and acute respiratory failure (12.6%). Hematological and lymphatic associations featured anemia (47.5%), thrombocytopenia (12.2%), and other conditions. Red blood cell transfusions were administered to 11.1% of patients. Conclusion: This study contributes valuable insights into CD, a rare and clinically heterogeneous disease. It underscores the importance of recognizing its associations and complications. Additionally, it highlights the need for further research and improved diagnostic and treatment guidelines to address the complexity of this condition. [ABSTRACT FROM AUTHOR]

The purpose is to ascertain the clinical impact of Castleman disease (CD) by reassessment of the real-world data from Peking University First Hospital (PKUFH). The results will contribute to the standardization of diagnosis and treatment on CDs. Based on the last 15-year retrospective real-world data from Peking University First Hospital (PKUFH), we reclassified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). A total of 44.1% (n = 52) of UCDs in our cohort were complicated with paraneoplastic pemphigus (PNP). The treatment of UCD is primarily surgical, with a 5-year overall survival (OS) of 88.1%. Patients with PNP had a poorer prognosis than those without PNP (82.9% (95% CI 123–178) vs 92.8% (95% CI 168–196), log-rank p = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143–213). Patients with UCD demonstrate a better overall prognosis than patients with MCD. But the prognosis of those complicated with PNP was poor. The differential diagnosis of MCD is extensive. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6–targeted drugs in China may contribute to the poor prognosis for patients with MCD. A preprint has previously been published (Guo et al. 34). [ABSTRACT FROM AUTHOR]

Early identification, diagnosis and treatment of TAFRO syndrome are very importants. We retrospectively analysed 6 patients with TAFRO syndrome. Their clinical manifestations, treatment methods, survival and other aspects were summarized. All patients were pathologically diagnosed with Castleman's disease, with fever, an inflammatory storm state and varying degrees of anasarca. All patients received steroid therapy; four of them also received chemotherapy, and 1 received rituximab. Of the 3 patients with severe disease, only 1 patient who received the recommended dose of glucocorticoids survived. Early administration of glucocorticoids can improve the prognosis, especially in patients with severe disease, and adequate glucocorticoids are important. [ABSTRACT FROM AUTHOR]

Castleman's disease (CD) is a rare and heterogeneous lymphoproliferative disorder, with limited available clinical information in Brazil. A retrospective study was carried out through information contained in the medical records of 51 patients, between July 1999 and June 2020. Seven patients were excluded, and 44 were analyzed in total. The average age of unicentric CD (UCD) patients was 35 years old and of multicentric CD (MCD) patients was 49 years old (p = 0.013). Regarding gender, there was a predominance of females among patients with UCD (68.4%) and males in patients with MCD (57.9%) (p = 0.103). The most common site of involvement in UCD was the cervical region (36.8%). A total of 73.7% of patients with UCD and 68.4% of patients with MCD presented the histological form hialyne-vascular (HV) (p = 0.499). Most patients with laboratory abnormalities had MCD. A total of 78% of the patients were asymptomatic, with the majority of symptomatic patients with MCD (p = 0.042). Only two of the 27 patients evaluated for the presence of human immunodeficiency virus (HIV) had positive serology. HHV-8 was evaluated in 14 cases, being positive in two. Of the patients with UCD, 94.7% underwent excisional biopsy, against only 41.2% of patients with MCD (p = 0.01). The mean follow-up was 61 months. We observed similarities in the clinical profile between patients in our study and patients described in the literature, such as gender, mean age, B symptoms, visceromegaly, fluid accumulation, and treatment. Unlike the literature, the cervical region was the most affected site, besides the greater association of the HV histological subtype among patients with MCD. [ABSTRACT FROM AUTHOR]

Here we present the case of a patient with simultaneous CD, solitary plasmacytoma with minimal bone marrow involvement, and POEMS syndrome followed by progression into multiple myeloma (MM). In CD without clonal plasma cell disorder, the Castleman disease variant of POEMS syndrome may occur and usually presents as a less severe and sensory polyneuropathy [[4]]. The diagnosis of POEMS syndrome requires the two mandatory criteria polyneuropathy and monoclonal plasma cell proliferation as well as one of the three other major criteria (Castleman disease [CD], sclerotic bone lesion, and elevated vascular endothelial growth factor [VEGF]) and one of the six minor criteria [[1]]. [Extracted from the article]

Background: Castleman disease affects lymph nodes with abnormal cell growth. It has unicentric (single node) Castleman disease (UCD) and multicentric (multiple nodes) Castleman disease (MCD) forms. MCD is systemic, with diverse symptoms, necessitating systemic treatment. Idiopathic MCD (iMCD) clinical subtypes are divided into iMCD- not otherwise specified (NOS) and iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticular fibrosis, organomegaly). UCD, iMCD-NOS, and iMCD-TAFRO mainly exhibit histopathology of hyaline vascular type, plasma cell type, and hyper vascular type, respectively. Case presentation: A 21-year-old female with no comorbidities presented to the outpatient department (OPD) with left inguinal swelling, gradually growing over four years, accompanied by fever and weight loss. Her past medical history included pulmonary TB 5 years prior and miscarriages. Vitals are within normal limits. Examination revealed a tender, nonreducible inguinal lump and a smaller neck swelling. Serological tests for infections were negative. Imaging revealed enlarged lymph nodes. Biopsy confirmed Castleman disease of the hyper vascular type. We performed surgical removal of the enlarged lymph nodes followed by close regular follow-up along with potential chemotherapy for relapse. Conclusion: Hyper vascular type of the lymph node histology in Idiopathic multicentric Castleman disease without TAFRO syndrome must be considered a differential diagnosis in lymphoproliferative disease. [ABSTRACT FROM AUTHOR]

Intrahepatic cholestatic jaundice caused by CD was suspected in a previous patient [[6]]. In our patients, IL-6 was also significantly elevated when intrahepatic cholestatic jaundice occurred and reduced to the normal range when jaundice disappeared. Here, we report 2 cases of idiopathic multicentric CD (iMCD) patients presenting with rare intrahepatic cholestatic jaundice which responded to bortezomib-based regimen. [Extracted from the article]

Although Castleman disease was first described in 1956, this disease includes various conditions, including unicentric Castleman disease with hyaline vascular histology, human herpesvirus-8 (HHV-8) related multicentric Castleman disease, idiopathic multicentric Castleman disease, and mimics of Castleman disease associated with other conditions. To date, Castleman disease remains incompletely understood due to its rareness and difficulties in clinical and pathological diagnosis. TAFRO syndrome was reported in Japan in 2010. Because lymph node histology is similar in patients with TAFRO syndrome and Castleman disease, TAFRO syndrome is described as a related disorder of Castleman disease. Clinically, however, these conditions differ markedly. Although elevated interleukin-6 (IL-6) expression is characteristic of Castleman disease, increased expression of IL-6 may occur in patients with other diseases, making elevated IL-6 unsuitable for differential diagnosis. Further understanding of these disorders requires the identification of novel disease-specific biomarkers. This review article therefore outlines the characteristics of Castleman disease and TAFRO syndrome. [ABSTRACT FROM AUTHOR]

This letter, published in the Annals of Hematology, presents a case report of a 39-year-old male with Unicentric Castleman's disease (UCD) presenting as amyloid A cardiac amyloidosis. The patient experienced symptoms such as fatigue, weakness, microcytic anemia, low serum albumin, albuminuria, hypergammaglobulinemia, and elevated NT-proBNP. Diagnostic tests revealed ventricular septal thickening, enlarged lymph nodes, and positive Congo red staining for amyloid deposition. The patient underwent surgical resection of the abdominal mass, leading to improvements in cardiac and renal involvement. The authors emphasize the importance of identifying the underlying condition causing AA amyloidosis for timely intervention. [Extracted from the article]

A lymph node biopsy of the neck suggested "reactive hyperplasia" and a transbronchial lung biopsy suggested "lymphocytic interstitial pneumonia." 1 A Lung CT presentations before and after treatment. a Before BCD treatment. b After 3 months of BCD treatment. c After BD maintenance treatment. d Before Dara treatment. e After Dara treatment. Dear Editor, A 56-year-old woman with an 8-year history of lymph node enlargement was admitted to our hospital in September 2021. [Extracted from the article]

In conclusion, PD1 blockade may be a therapeutic choice for ALL/lymphoblastic lymphoma relapsing after allo-HSCT, if conventional options are neither available nor feasible. Relapses of haematological malignancies after allo-HSCT reported to respond to PD1 blockade included Hodgkin lymphoma [[5]-[10]], anaplastic large cell lymphoma [[11]], posttransplant lymphoproliferative disease [[12]], acute myeloid leukaemia [[14]] and ALL [[15]]. 1:CAS:528:DC%2BC2sXht12ks7c%3D. 10.1007/s12185-017-2181-9 10 Minson A, Douglas G, Bilmon I, Grigg A. Low dose PD-1 inhibition in relapsed refractory Hodgkin lymphoma after allogeneic stem cell transplant with concomitant active GVHD. PD-1 blockade for relapsed lymphoma post-allogeneic hematopoietic cell transplant: high response rate but frequent GVHD. [Extracted from the article]

Post-transplant lymphoproliferative disorders (PTLD) exclusively affecting the central nervous system—primary CNS-PTLD (pCNS-PTLD)—are rare. There is no standard therapy, and previous case series have included heterogeneous treatment approaches. We performed a retrospective, multi-centre analysis of 14 patients with pCNS-PTLD after solid organ transplantation (SOT) treated in the prospective German PTLD registry with reduction of immunosuppression (RI), whole-brain radiotherapy (WBRT), and concurrent systemic rituximab between 2001 and 2018. Twelve of fourteen patients were kidney transplant recipients and median age at diagnosis was 65 years. Thirteen of fourteen cases (93%) were monomorphic PTLD of the diffuse large B-cell lymphoma type, and 12/13 were EBV-associated. The median dose of WBRT administered was 40 Gy with a median fraction of 2 Gy. The median number of administered doses of rituximab (375 mg/m2) IV was four. All ten patients evaluated responded to treatment (100%). Median OS was 2.5 years with a 2-year Kaplan–Meier estimate of 63% (95% confidence interval 30–83%) without any recorded relapses after a median follow-up of 2.6 years. Seven of fourteen patients (50%) suffered grade III/IV infections under therapy (fatal in two cases, 14%). During follow-up, imaging demonstrated grey matter changes interpreted as radiation toxicity in 7/10 evaluated patients (70%). The combination of RI, WBRT, and rituximab was an effective yet toxic treatment of pCNS-PTLD in this series of 14 patients. Future treatment approaches in pCNS-PTLD should take into account the significant risk of infections as well as radiation-induced neurotoxicity. [ABSTRACT FROM AUTHOR]

Germinotropic lymphoproliferative disorder is a rare and rather enigmatic novel entity with distinctive clinicopathological features, one of which is the typical co-infection by Human herpesvirus 8 and Epstein-Barr virus. Human herpesvirus 8 is a lymphotropic virus detected in Kaposi sarcoma, multicentric Castleman disease, primary effusion lymphoma, Human herpesvirus 8-positive diffuse large B cell lymphoma not otherwise specified, and germinotropic lymphoproliferative disorder. Co-infection by Human herpesvirus 8 and Epstein-Barr virus is identified only in two lymphoproliferative diseases: germinotropic lymphoproliferative disorder and primary effusion lymphoma, which are otherwise diseases with totally different clinical presentations and outcomes. Unlike primary effusion lymphoma mostly occurring in immunocompromised individuals and following an aggressive course, germinotropic lymphoproliferative disorder usually presents with single or multiple lymphadenopathy affecting mainly immunocompetent individuals and mostly follows an indolent course. Based on the PRISMA guidelines, we carried out a systematic search on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and Cochrane Library using the search terms "germinotropic" and "lymphoproliferative disorder." Current scientific literature reports just 19 cases of germinotropic lymphoproliferative disorder. The purpose of our systematic review is to improve our understanding of the disease, focusing on epidemiology, clinical presentation, pathological features, treatment, and outcome. In addition, we discuss the differential diagnosis with the other Human herpesvirus 8-related lymphoproliferative diseases as currently recognized in the World Health Organization classification, adding a focus on lymphoproliferative disorders showing overlapping features. [ABSTRACT FROM AUTHOR]

1 Fundus photographs and magnetic resonance imaging (MRI) of a patient with EBV-positive posttransplant lymphoproliferative disease masked by initial CMV retinitis. a Fundus photograph on presentation, showing presence of retinitis (arrow), retinal haemorrhages in inferior retina and disc swelling. Histopathological examination finally revealed the diagnosis of EBV-positive B cell PTLD, masked by CMV retinitis on initial presentation. 2 Epstein Barr virus (EBV) DNA and histopathologic features of a patient with EBV-positive posttransplant lymphoproliferative disease masked by initial CMV retinitis. a Quantification of intravitreal (red line), plasma (blue line) and cerebrospinal fluid (CSF, green) EBV DNA. [Extracted from the article]

Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China. The novel diagnostic criteria for iMCD were applied, and POEMS syndrome, IgG4-related diseases, and follicular dendritic cell sarcomas cases were excluded from analyses. Total response rate of rituximab- and cyclophosphamide-containing regimens was 55.5%, with 33.3% (9/27) of the cases reaching CR and 22.2% (6/27) PR. In the 14 cases of R-R iMCD, total response rate was only 42.9% (CR 14.3% [2/14], PR 28.6% [4/14]). The 5-year OS of these 27 iMCD cases was 81% (95% CI 64-98; 27 total patients, 4 events, 23 censored) after receiving these regimens, but the 5-year PFS was 43% (95% CI 19-66; 25 total patients, 11 events, 14 censored). Thus, rituximab-based regimens should be considered for the treatment of iMCD patients when siltuximab is not available and potentially in siltuximab-refractory cases. [ABSTRACT FROM AUTHOR]

Castleman's disease (CD) is a rare lymphoproliferative disorder, and its prevalence in Thailand is not known. This 10-year period study investigated the prevalence of CD in Thailand, and the clinical characteristics and outcomes of Thai CD patients, with special focus on the existence and prevalence of TAFRO syndrome. TAFRO syndrome is defined as CD with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thirty-three CD patients diagnosed and treated at Siriraj Hospital during January 2007 to December 2016 were included. The prevalence of CD was 1.4 per 1,000,000 patients/10 years. Median age was 46 years, with slight female predominance. Six patients were assigned to the TAFRO group. A high proportion of TAFRO syndrome (18.2%) was found among Thai adult CD patients. In addition to routine TAFRO diagnostic criteria, significantly lower hemoglobin and albumin levels were observed in the TAFRO group than in the non-TAFRO group. Treatment outcomes of CD patients were complete remission (52%), stable disease (30%), and death (13%). Three-year overall survival in the non-TAFRO group and TAFRO group was 88 and 50%, respectively. While most CD patients had a good prognosis, severe cases with TAFRO syndrome had poor outcome. [ABSTRACT FROM AUTHOR]

TAFRO syndrome was first described as a variant of multicentric Castleman's disease with thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly. We report the case of a 25-year-old Caucasian male with diagnosis of TAFRO syndrome and present a literature review. The objective of the study was to compare TAFRO syndrome between Japanese and non-Japanese patients. Cases were included by searching the term "TAFRO" in the Medline database using PubMed between 2010 and 2016. The Student t test and Mann-Whitney U test were used to compare continuous variables. Fisher's exact test was used for categorical variables. Statistical significance was set at p < 0.05. Forty-four cases were included. Thirty-two patients (73%) were of Japanese origin. Japanese patients were significantly older than non-Japanese ones (52.0 ± 13.6 years versus 36.9 ± 19.8 years, p = 0.0064) but there was no difference in gender. Creatinine level on admission was significantly higher in the non-Japanese group (1.87 ± 0.84 mg/dL versus 1.32 ± 0.57 mg/dL, p = 0.0347). There were no significant differences concerning lymphadenopathy, elevated number of megakaryocytes on bone marrow aspiration, autoimmune abnormalities, and the following parameters on admission: platelet count, hemoglobin, albumin, alkaline phosphatase (ALP). Corticotherapy was always used on induction for Japanese patients while it was only used in 75% of the cases on induction in non-Japanese patients (p = 0.0166). Our study was the first to compare TAFRO syndrome according to ethnicity. Japanese patients were significantly older and had a significantly lower creatinine level on admission than non-Japanese patients. [ABSTRACT FROM AUTHOR]

1:CAS:528:DC%2BC3MXltlWnsg%3D%3D. 10.1136/jcp.2010.082958 8 Zongfei J, Lijuan Z, Ying S, Dongmei L, Sifan W, Xiufang K, et al. (2021) Improved clinical outcomes of tocilizumab versus cyclophosphamide for IgG4-related disease: insights from a prospective IgG4-related disease registry. A recent prospective cohort study demonstrated excellent response to tocilizumab using fixed 6-month duration therapy in 14 treatment-naïve and refractory IgG4-RD patients, including 50% complete response [[8]]. Dear Editor, IgG4-related disease (IgG4-RD) is an immune-mediated condition characterized by tissue infiltration by IgG4-secreting plasma cells [[1]]. [Extracted from the article]

Plasmablasts were co-infected by human herpesvirus 8 (HHV-8) and Epstein-Barr virus (EBV), being positive for HHV-8 (Fig. 1Clusters of large atypical cells colonizing germinal centers (HE 20x); inset left HHV-8 positive staining of plasmablasts; inset right Epstein-Barr virus positivity of plasmablasts by in situ hybridization (EBER) Clusters of plasmablasts show a predilection to colonize GC, hence the definition germinotropic, and are co-infected by HHV-8 and EBV [[1]-[10]]. MCD is HHV-8-positive and always EBV-negative, differently from GLD which is co-infected by HHV-8 and EBV [[1]-[10]]. [Extracted from the article]

Both multicentric Castleman's disease (MCD) and immunoglobulin (Ig)G4-related disease (IgG4-RD) are systemic diseases, presenting with hypergammaglobulinemia and elevated serum levels of IgG4. However, with regard to histopathological findings, MCD shows atrophic germinal centers. On the other hand, expanded germinal centers are detected in IgG4-RD. We extracted germinal centers from specimens of each disorder by microdissection and analyzed the expression of mRNAs by real-time polymerase chain reaction to clarify the mechanisms underlying atrophied germinal centers in MCD. This analysis disclosed loss of interleukin (IL)-21 and B cell lymphoma (Bcl)-6 in the germinal centers of MCD. Loss of IL-21 is considered to be involved in the disappearance of Bcl-6 and leads to atrophied germinal centers in MCD. [ABSTRACT FROM AUTHOR]

Considering that regulatory T cells may support lymphoma progression, while Th17 cells inhibit tumor growth in B-cell lymphoma [[7]], IGU potentially promotes the relapse of MTX-LPD. Dear Editor, Iguratimod (IGU) is a novel antirheumatic immunomodulatory drug [[1]], and its impact on the development of lymphoproliferative disorder (LPD) remains unclear. [Extracted from the article]

It confirms that complement inhibitors are effective therapeutic weapons for CA-mediated AIHA complicating lymphoproliferative disorders, even after failed etiologic B-cell therapies, for refractory or relapsed patients. Eculizumab, a real-life successful treatment for refractory cold agglutinin-mediated auto-immune hemolytic anemia secondary to lymphoproliferative disorders Dear Editor, Cold agglutinin (CA)-mediated auto-immune hemolytic anemia (AIHA) is characterized by chronic hemolysis, most often due to a lymphoproliferative disorder. [Extracted from the article]

The article presents six cases of patients with idiopathic multicentric Castleman's disease (iMCD) with negative HIV serology and no replicating HHV-8 virus. Long-term intravenous tocilizumab was administered to all patients, which showed significant improvement of hyperimmunoglobulinemia. Lifelong administration of tocilizumab is suggested to manage disease activity, noting the observed recurrence of the case after cessation of tocilizumab.

Castleman's disease (CD) is a benign lymphoproliferative disorder characterized by dysfunctional lymphatic node hyperplasia. Lymphatic node hyperplasia is associated with elevated levels of inhibitor of differentiation 1 (ID1) in many human tumors. To assess the possible role of ID1 expression as a prognostic marker in multicentric CD (MCD), intra-lymph node ID1 expression was analyzed and related to clinical characteristics and outcomes in 48 patients. Furthermore, the correlation between ID1 and possible signaling molecules such as interleukin-6 (IL6), phosphorylated extracellular response kinase (p-ERK), and vascular endothelial growth factor C (VEGFC) was explored on six fresh MCD surgical specimens. Immunohistochemistry revealed that the patients with extensive ID1 expression had significantly poorer prognosis, compared to those with localized ID1. In addition, ID1 was positively associated with levels of IL6, p-ERK, and VEGFC. We conclude that ID1 may ultimately be a prognostic marker in MCD and that the IL6/ERK/VEGFC pathway is involved in the progress of this disease. [ABSTRACT FROM AUTHOR]

Castleman's disease (CD) is a rare non-neoplastic lymphoproliferative disorder with ambiguous etiology. This study aimed to evaluate the potential association between hepatitis B virus (HBV) and CD and to characterize the HBV-positive CD patients in China, an endemic area for HBV infection. We compared the prevalence of HBV infection in 35 consecutive CD patients initially diagnosed in our hospitals over a 10-year period with an age- and sex-matched healthy control, a national population-based control, and a non-Hodgkin's lymphoma (NHL) control. We found that the prevalence of HBV infection in CD was 17.1% (6/35), which was as high as the NHL control (19.9%, P = 0.693), but significantly higher than the age- and sex-matched healthy control (6.9%, P = 0.033) and the national population-based control (7.2%, P = 0.037). Next, we compared the clinicopathological characteristics between HBV-positive and HBV-negative CD patients. We found that HBV-positive CD patients had a significantly higher NHL malignant transformation rate (33.3% vs. 0%, P = 0.025), higher splenomegaly rate (83.3% vs. 27.6%, P = 0.019), and much poorer prognosis (estimated mean overall survival time (50.83 vs. 64.34 months, P = 0.016). In conclusion, our findings suggest an association between HBV infection and development of CD. CD patients with HBV infection have their own distinguished features. [ABSTRACT FROM AUTHOR]

POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. This study reviewed the clinical characteristics and long-term outcome of 99 consecutive Chinese patients with newly diagnosed POEMS syndrome in a single institute. The median age of 99 patients was 45 years, and the ratio of men/women was 1.4. The median time from onset of symptoms to diagnosis was 18 months. The typical five features of peripheral neuropathy, organomegaly, endocrinopathy, M protein, and skin change remained to be essential for patients with POEMS syndrome in China. The unusual features like pulmonary hypertension (36%) and renal impairment (37%) were not uncommon in China. Eighty-three percent patients were alive after follow-up time of 25 months, and 10% patients had survived more than 60 months. Melphalan-based therapy (OR, 0.076; 95% CI, 0.02-0.285) and normal renal function (OR, 0.246; 95% CI, 0.076-0.802) were independent prognostic factors for the survival of patients with POEMS syndrome. In conclusion, POEMS syndrome in Chinese patients was a multi-systemic disease with clinical features similar to non-Chinese ones. Active therapy can effectively improve the prognosis of patients with POEMS syndrome. [ABSTRACT FROM AUTHOR]

Based on the pathology and prognosis of other DCLDs and experience gained from our patient, we inferred that effective treatment might delay the progression of lung cysts but be incapable of reversing them. The treatment goals of these patients should be to improve symptoms and control inflammation rather than striving to reverse lung cysts. [Extracted from the article]

We report a case of Castleman’s disease that developed tumor lysis syndrome spontaneously and after systemic chemotherapy. A 44-year-old male patient was admitted with a 2-week history of abdominal distension accompanied by dyspnea. Physical examination revealed multiple lymph node enlargements. After admission, spontaneous hemoperitoneum developed and he underwent exploratory laparotomy, with the removal of the ruptured spleen. Pathologic review of the splenic tissue and excised lymph node gave the diagnosis of multicentric Castleman’s disease. He experienced two episodes of tumor lysis syndrome, initially spontaneous and then chemotherapy related, which needed vigorous management including hemodialysis and intensive fluid therapies. To our knowledge, this is the first reported case of Castleman’s disease complicated by tumor lysis syndrome. This suggests that the possibility of tumor lysis syndrome should be considered when treating Castleman’s disease with a large disease burden. [ABSTRACT FROM AUTHOR]

It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD – plasma cell type – in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course – even with therapy – in others. Still, effective treatment strategies need to be developed. [ABSTRACT FROM AUTHOR]

Systemic mastocytosis is a rare condition characterized clinically by the local consequences of vasoactive peptides released from infiltrating mast cells in the reticuloendothelial tissues. Mast cells originate from the pluripotent bone marrow stem cells; it is therefore not surprising that myeloproliferative and myelodysplastic disorders commonly coexist or terminate the clinical phase of mastocytosis. We report here, to our knowledge, the first case of Hodgkin's and Castleman's disease occurring in a patient with co-existent systemic mastocytosis, which remained unchanged after combination chemotherapy for Hodgkin's disease. [ABSTRACT FROM AUTHOR]

A patient is presented who had Castleman's disease with constitutional symptoms, a palpable supraclavicular/ axillar mass, and a microcytic anemia, among other laboratory abnormalities, including elevated levels of interleukin-6. Treatment consisted of irradiation of the involved area, with subsequent disappearance of all symptoms and normalization of the laboratory abnormalities. Iron kinetic studies demonstrated a hypoproliferative erythropoiesis, which normalized after radiotherapy. Hypoproliferative erythopoiesis could not be ascribed to serum inhibitors, since normal burst-forming units were observed in the absence or presence of autologous serum. The role of interleukin-6 in relation to Castleman's disease is highlighted. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses the case of a 50 year old male suffering with Multicentric Castleman disease (MCD) accompanied by systemic manifestations such as Autoimmune hemolytic anemia (AIHA) with wIgM and IgG antibodies.

The article presents a case study of a 49-year-old male patient who was diagnosed with depressive disorder and dysmyelinating peripheral polyneuropathy with asthenia, anorexia, generalized edema, and diarrhea. In this study, the efficacy of bortezomib being applied in refractory form of multicentric Castleman's disease associated to poems syndrome is examined. It discusses the pathological aspects of Castleman's disease which is said to be a rare lymphoproliferative disorder.

We describe one patient with localized Castleman's disease (CD) of the mixed hyaline vascular and plasma cell type located at the mesentery of the small bowel, associated with systemic amyloidosis and nephrotic syndrome. A true nephrotic syndrome has rarely been reported in patients with CD. In the literature, it has been suggested that clinical and laboratory manifestations generally improved after surgical resection of the tumor. However, in our case, clinical and laboratory findings did not regress after operation followed by colchicine therapy. [ABSTRACT FROM AUTHOR]