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Chronic myelogenous leukemia at blast crisis with a T-cell phenotype (T-ALL CML-BC) at diagnosis, without any prior history of CML is extremely rare. After the introduction of tyrosine kinase inhibitors (TKIs), CML patients have a median survival comparable to general population and accelerated/blast crisis are rarely encountered. Most CML patients (80%) transform into acute myeloid leukemia and the rest into B-ALL. Anecdotal cases of Ph+ T-ALL, either de novo or in the context of CML-BC have been reported. Left shift in the blood, the presence of splenomegaly/extramedullary infiltration and the occurrence of BCR::ABL1 rearrangement in both the blastic population, as well as in the myeloid cell compartment are key points in differentiating de novo Ph+ T-ALL from T-ALL CML-BC. The latter is a rare entity, characterized by extramedullary disease, p210 transcript and clonal evolution. Lack of preceding CML does not rule out the diagnosis of T-ALL CML-BC. Prompt TKI treatment with ALL-directed therapy followed by allogeneic stem cell transplantation may offer long-term survival in this otherwise poor prognosis entity. In this paper, we describe a patient with T-ALL CML-BC at presentation, still alive 51 months after diagnosis and we offer a review of the literature on this rare subject. All clinical and laboratory features are provided in order to distinguish de novo Ph+ T-ALL from T-ALL CML-BC, underscoring the prognostic and therapeutic significance of such a differentiation. [ABSTRACT FROM AUTHOR]

Abstract: Extracorporeal photopheresis (ECP) is an established therapy for cutaneous T-cell lymphoma (CTCL). The objective of this study was to further explore the clinical efficacy of ECP combined with immunomodulatory agents. Eighteen patients with histologically proven CTCL were followed-up after therapy with ECP, mainly combined with interferon-α or bexarotene. A total of 61% of patients responded to therapy (n =11; CR: 5, PR: 6). Median survival was 51months, progression free survival was 28months and response duration was 29±23.9months. ECP combined therapy was highly effective or had a palliative effect in CTCL resistant to previous treatments. [Copyright &y& Elsevier]

Primary cutaneous marginal zone lymphoma (PCMZL) is an indolent disease. Treatment options include excision, local irradiation, interferon- α or chemotherapy. We present two patients with PCMZL and multiple skin lesions successfully treated with intralesional administration of the anti-CD20 monoclonal antibody rituximab. The first presented with four red skin lesions and the second with two. Biopsy of the largest lesion revealed marginal zone B-cell lymphoma in both patients. There was no evidence of systemic involvement in either patient. Both patients were treated with intralesional rituximab for 18 consecutive weeks. Skin lesions gradually regressed. Apart from mild local pain during the injection, no other adverse effects were observed. In conclusion, rituximab can be safely administered intralesionally in patients with PCMZL and can produce disease remission. [ABSTRACT FROM AUTHOR]

ATLL is etiologically associated with HTLV-I retrovirus. A population of 10 to 20 million worldwide is estimated to be infected by the virus, but only 1 - 4% develop ATLL during a 70-year lifespan. The latency period is more than 30 years. The aim of this study was to report two cases of ATLL in Greek patients with the concomitant study of their family members. A 55-year-old woman and a 59-year-old man presented with leucocytosis and lymphocytosis. Both were asymptomatic and physical examination was unremarkable except for minimal lymphadenopathy in the second patient. In both patients blood smears showed small-to-medium-sized, multilobulated lymphocytes, with different degrees of nuclear irregularity. Immunophenotypic study was as follows: CD2 + (97%), CD3 + (95%), CD5 + (95%), CD3/CD4 + (93%), CD3/CD25 + (84%), CD7 - /CD4 + (89%) CD2 + /HLA-DR + (53%), TCRaβ + (96%) and CD7-(7%). Bone marrow biopsy revealed a normal cellularity with dyserythropoiesis and scattered small lymphocytes (CD4 + on immunostaining) Serum HTLV I and II antibodies were positive. T-cell receptor γ-chain rearrangement was positive in blood lymphocytes by PCR. Cytogenetic analysis showed complex karyotypic abnormalities. DNA analysis by PCR demonstrated the integration of the HTLV-1 DNA in the DNA of the neoplastic T cells. Both patients rapidly developed acute type ATLL. In the first patient multiple subcutaneous nodules on the palmar surface of both hands were also observed. She received deoxycoformycin, which was stopped because of autoimmune hemolytic anemia. Corticosteroid treatment was initiated, with gradual improvement. She suffered from recurrent opportunistic infections. She is currently under interferon and zidovudine therapy with stable blood parameters. Chemotherapy was administered to the other patient with > 50% initial response. Both patients' families were tested for serum anti HTLV-I antibodies and their mates were found to be positive; they also had detectable viral DNA by PCR analysis while asymptomatic, with no abnormal clinical findings and normal white blood cell count and morphology. In conclusion, the two aforementioned patients are the first fully documented ATLL patients described in Greece. Investigation for HTLV-I antibodies should be mandatory in all patients with T-cell lymphoproliferative disorders. [ABSTRACT FROM AUTHOR]

Determines the efficiency of recombinant human erythropoietin in the restoration of disease-related anemia in B-chronic lymphoproliferative disorders. Demonstration of whether the correction is related to the serum erythropoietin levels before treatment; Comparison of the degree of response with patients' clinical status.

Simple Summary: The significance of serum beta-2 microglobulin (sβ2m) in Hodgkin lymphoma (HL) is controversial. In an effort to investigate the prognostic significance of sβ2m levels in a large series of patients with HL, we analyzed 915 patients, who were treated with ABVD or equivalent regimens with or without radiotherapy. Sβ2m levels were measured by a radioimmunoassay (upper normal limit 2.4 mg/L). The median sβ2m levels were 2.20 mg/L. Freedom from progression (FFP) was significantly inferior in patients with a higher sβ2m at all tested cutoffs. The best cutoff was 2.0 mg/L (10-year FFP 83% vs. 70%, p = 0.001), which performed better than the 2.4 mg/L cutoff ("normal versus high"). Our data suggest that higher sβ2m is a significant independent predictor of FFP, OS and HLSS in HL but the optimal cutoff appears to lie within the normal limits in this predominantly young patient population. The significance of serum beta-2 microglobulin (sβ2m) in Hodgkin lymphoma (HL) is controversial. We analyzed 915 patients with HL, who were treated with ABVD or equivalent regimens with or without radiotherapy. Sβ2m levels were measured by a radioimmunoassay (upper normal limit 2.4 mg/L). Sequential cutoffs (1.8–3.0 by 0.1 mg/L increments, 3.5 and 4.0 mg/L) were tested along with ROC analysis. The median sβ2m levels were 2.20 mg/L and were elevated (>2.4 mg/L) in 383/915 patients (41.9%). Higher sβ2m was associated with inferior freedom from progression (FFP) at all tested cutoffs. The best cutoff was 2.0 mg/L (10-year FFP 83% vs. 70%, p = 0.001), which performed better than the 2.4 mg/L cutoff ("normal versus high"). In multivariate analysis, sβ2m > 2.0 mg/L was an independent adverse prognostic factor in the whole patient population. In multivariate overall survival analysis, sβ2m levels were predictive at 2.0 mg/L cutoff in the whole patient population and in advanced stages. Similarly, sβ2m > 2.0 mg/L independently predicted inferior HL-specific survival in the whole patient population. Our data suggest that higher sβ2m is an independent predictor of outcome in HL but the optimal cutoff lies within the normal limits (i.e., at 2.0 mg/L) in this predominantly young patient population, performing much better than a "normal versus high" cutoff set at 2.4 mg/L. [ABSTRACT FROM AUTHOR]

Yet, the description of a polymorphic cell infiltration in LN biopsy, the absence of clinical lymphadenopathy at presentation, the history of psoriatic arthritis as well as the preceded treatment with anti-tumor necrosis factor (TNF)- and cyclosporine raised diagnostic concerns. Although the patient could not finally avoid R-CHOP immunochemotherapy, this case highlights that a trial of NBA withdrawal under very close monitoring could be a rational therapeutic choice even in patients with aggressive ID-LPDs. Keywords: diffuse large B-cell lymphoma; psoriasis; infliximab; apremilast; novel biologic agent; anti-TNF EN diffuse large B-cell lymphoma psoriasis infliximab apremilast novel biologic agent anti-TNF 1695 1700 6 10/12/23 20231001 NES 231001 The association of psoriasis with cancer has been a matter of debate, especially in the era of the novel biologic agents (NBA). Lastly, taking into consideration our case with the re-appearance of the lymphoma despite the change of NBA as well as all the other cases described in Table 1, the appearance of LPDs in psoriatic patients is a more complex process influenced by clinical disease duration and extension, the time length of immunosuppression and the nature of the various treatments administered. [Extracted from the article]

Dose-adjusted EPOCH-R compared with R-CHOP as frontline therapy for diffuse large B-cell lymphoma: clinical outcomes of the phase III intergroup trial alliance/CALGB 50303. The latter do not carry concurrent c-myc/bcl-2 and/or bcl-6 rearrangements and usually have the morphology of HGBL with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (HGBL-DLBCL/BL). Dear Editor, In the 2016 WHO classification, HGBL was defined as a separate entity, classified into double/triple-hit lymphomas and HGBL not otherwise specified (HGBL-NOS). [Extracted from the article]

Kikuchi-Fujimoto disease is a form of reactive lymphadenopathy, which was firstly described in Japan, but is uncommon in the Western world. We retrospectively reviewed the medical records of nine cases of adult or adolescent Kikuchi’s disease diagnosed in a single Haematology Unit in Athens, Greece between 1990 and 2006. The median age of the patients was 25 years (14–40) and 8/9 were females. All patients presented with cervical lymphadenopathy sparing the supraclavicular fossa; one had associated axillary lymphadenopathy, seven had fever and two were asymptomatic. The median duration of lymphadenopathy before presentation was 30 days (10–45). Just palpable splenomegaly was recorded in three patients. The median value of the maximal lymph node diameter was 2 cm (1–5) and only 1/9 had nodes >2 cm in their largest diameter. Lymphadenopathy was tender in two patients; hard nodes were observed in three patients. The median leukocyte count was 4.7 × 109/l (2.2–4.9) with a normal differential in 7/9 patients. No infectious agent could be demonstrated. One patient had clinical and laboratory evidence of primary antiphospholipid syndrome (APLS). In conclusion, Kikuchi’s disease represents a rare but important diagnostic possibility for patients presenting with lymphadenopathy in Greece and other western countries. In this setting, autoimmune disorders, mainly lupus and APLS, should be considered and excluded by the appropriate laboratory work-up. [ABSTRACT FROM AUTHOR]

Purpose: To present our long-term experience regarding the use of chemotherapy plus low-dose involved-field radiotherapy (IFRT) for clinical Stage I-IIA Hodgkin''s lymphoma.Methods and materials: We analyzed the data of 368 patients. Of these, 66 received mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) and 302 received doxorubicin (or epirubicin), bleomycin, vinblastine, and dacarbazine [A(E)BVD]. Patients with complete remission or very good partial remission were scheduled for low-dose IFRT (≤3200 cGy).Results: The 10-year failure-free survival (FFS) and overall survival (OS) rate was 85% and 86%, respectively. A(E)BVD-treated patients had superior 10-year FFS and OS rates compared with MOPP-treated patients (87% vs. 75%, p = 0.009; and 93% vs. 71%, p = 0.0004, respectively). Only 10 of 41 relapses had any infield (irradiated) component. Of the complete responders/very good partial responders treated with low-dose IFRT, those who received <2800 cGy had inferior FFS but similar OS as those who received 2800–3200 cGy. Adverse prognostic factors for FFS included age ≥45 years, leukocytosis ≥10 × 109/L, and extranodal extension. Secondary acute leukemia developed after MOPP with or without salvage therapy (n = 6) or after ABVD plus salvage therapy (n = 2). None of the nine secondary solid tumors developed within the RT fields.Conclusion: IFRT at a dose of 2800–3000 cGy is highly effective in clinical Stage I-IIA HL patients who achieved a complete response or very good partial response with A(E)BVD. The long-term toxicity with respect to secondary malignancies appears to be acceptable. [Copyright &y& Elsevier]

Kyrtsonis M-C, Vassilakopoulos TP, Siakantaris MP, Kokoris SI, Gribabis DA, Dimopoulou MN, Angelopoulou MK, Pangalis GA. Serum syndecan-1, basic fibroblast growth factor and osteoprotegerin in myeloma patients at diagnosis and during the course of the disease. Eur J Haematol 2004: 72: 252–258. © Blackwell Munksgaard 2004. Neovascularisation and bone resorption are related to myeloma disease activity. To investigate the possible prognostic importance of serum syndecan-1, basic fibroblast growth factor (bFGF) and osteoprotegerin (OPG) levels, the relationship between them, with parameters of disease activity and the effect of treatment on their levels. Twenty-seven patients were studied from diagnosis and an additional five from remission, for a median follow-up of 40 months. Twenty-three patients received chemotherapy plus bisphosphonates and nine only bisphosphonates. Sera from 11 healthy individuals (HI) were used as controls. Cytokines were determined by commercially available enzyme-linked immunosorbent assays (ELISA) kits. In HI, median syndecan-1 was 40 ng/mL (28–75), bFGF 8 pg/mL (7–30), OPG 35 pg/mL (4–100). Pretreatment median serum syndecan-1 was 177.5 ng/mL (34–3500), bFGF 11.5 pg/mL (8–65) and OPG 100 pg/mL (4–1000). Pretreatment syndecan-1, bFGF and OPG serum levels were increased in patients compared with HI ( P = 0.001, 0.03 and 0.01, respectively). Syndecan-1 and bFGF levels were correlated with stage ( P = 0.004 and 0.03, respectively). Both syndecan-1 and OPG levels were correlated with β2M ( P = 0.04 and 0.01, respectively). Patients with elevated syndecan-1 and bFGF serum levels had shorter survival than patients with normal levels ( P = 0.01 and 0.05, respectively). After chemotherapy syndecan-1 and OPG levels were found to be decreased in responders and syndecan-1 level was reduced in patients receiving bisphosphonates alone. Pretreatment syndecan-1, bFGF and OPG levels were found to be increased at diagnosis. Syndecan-1 and OPG fluctuated according to MM activity. Elevated serum syndecan-1 and bFGF levels predicted short survival. [ABSTRACT FROM AUTHOR]

Abstract: Objectives : To investigate the prognosis of patients with Hodgkin's lymphoma (HL) who relapse following a complete remission (CR) achieved by chemotherapy with or without radiotherapy (CT±RT), and to identify prognostic factors for freedom from second progression (FF 2 P). Methods : We analyzed the prognostic significance of the initial CT regimen (4 vs. 7–8 drugs), treatment-free interval (TFI), and demographic, clinical, and laboratory factors at the time of relapse and diagnosis, in 113 patients with HL, who relapsed after a CR achieved by CT±RT. Results : Conventional salvage CT±RT was administered in 107 patients, while six received RT only. The 5-yr FF 2 P was 24%, while the 10-yr survival after relapse (O 2 S) was 39% and was not afffected by the initial CT regimen. Multivariate analysis revealed that extranodal disease at relapse ( P <0.001), TFI<6 month ( P <0.001), >=5 involved sites at diagnosis ( P =0.04) and anemia at relapse ( P =0.03) were independent predictors of FF 2 P. 55% of patients had 0 or 1 of these adverse prognostic factors. The 5-yr FF 2 P of patients with 0, 1 or 2 adverse factors was 58%, 34% and 5% ( P <0.0001). The corresponding rates for 10-yr O 2 S were 68%, 51% and 25%, respectively ( P =0.002). Conclusions: Our data confirmed the significance of TFI and extranodal relapse and demonstrated a potential role for anemia at relapse and number of involved sites at diagnosis, for the prognosis of patients with HL relapsing after CT±RT. The combination of these prognostic factors defines a sizeable subgroup of patients with favorable outcome following conventional salvage therapy. [ABSTRACT FROM AUTHOR]

Reports on a patient with primary non-Hodgkin's lymphoma of the gallbladder. Diagnosis of the patient as a T-cell lymphoblastic lymphoma; Inclusion of surgery and postoperative chemotherapy as part of treatment modalities; General prognosis for the disease; Achievement of complete remission.

Evaluates the efficacy of Epirubicin, Bleomycin, Vinblastine and Decarbazine (EBVD) combination chemotherapy followed by low dose involved field radiation therapy in patients with clinical stage I-IIA Hodgkin's disease. Dose administration; Survival rate of patients; Percentage of reduction of tumor load; Response rate.

The article discusses the treatment of splenic marginal zone lymphoma (SMZL) and the lack of randomized trials, highlighting the use of rituximab infusions for the treatment. It examines the bone marrow for complete response (CR), the impact of rituximab on the disease progression, and the overall survival (OS) rate.

The optimal treatment approach for patients with mantle cell lymphoma (MCL) is not well defined. Intensive therapeutic regimens result in high response rates and prolonged progression-free survival but at the expense of significant toxicity. We report here our results of the administration of rituximab plus chlorambucil (R-Chl) as first line treatment in patients with MCL. Twenty consecutively diagnosed patients were treated with this combination in which an induction and a maintenance arm were included. During induction, rituximab was administered at a dose of 375 mg/m2 on day 1, while chlorambucil was given afterward at a dose of 10 mg/day for 10 consecutive days for eight cycles and then as a single agent for an additional four cycles. Maintenance consisted of rituximab administration every 2 months for 1 year. Most patients had indolent disease features such as a low mantle-cell international prognostic index (MIPI) score. The overall response rate was 95% (90% CR, 5% PR). Among patients in CR, 78% presented a molecular remission. The 3-year progression-free survival was 89%. There were no serious side effects. These results show that the R-Chl combination could be an effective therapeutic option as first line treatment in MCL, especially for patients with indolent disease characteristics. [ABSTRACT FROM AUTHOR]

In the present study, we assessed the clinical and pathological data of 76 patients with the diagnosis of non-gastric extranodal marginal zone B-cell lymphoma. The most commonly affected sites were salivary glands, skin, ocular adnexa, lung, intestine and Waldeyer's ring. Ann Arbor stage I disease was present in 39 patients (51%), stage II in 10 (13%) and stage IV in 27 (36%). In 17 cases (21%), the lymphoma presented at multiple mucosal sites. Lymph node and bone marrow involvement were present in 21% and 16%, respectively. Most cases were in the low or low-intermediate risk group. Treatment was heterogeneous and included chlorambucil in 59% either alone or in combination with other agents. Complete and partial remission was achieved in 79% and 7%, respectively, with an overall response rate of 86%. The 5- and 10-year overall survival and cause-specific survival rates were 94%, 82% and 95%, 91%, respectively. The 5- and 10-year progression free survival was 56% and 41%, respectively. The only feature associated with inferior outcome was disease localisation to the lung. [ABSTRACT FROM AUTHOR]

The prognostic value of baseline serum free light chain ratio (sFLCR) was investigated in 94 multiple myeloma (MM) patients. sFLCR was calculated as κ/ λ or λ/ κ, depending on the patients’ dominating monoclonal light chain. Median baseline sFLCR was 3·57 in κ-MM patients, 45·09 in λ-MM. ‘High’ sFLCR (≥ the observed median value for κ- and λ-MM respectively) correlated with elevated serum creatinine and lactate dehydrogenase, extensive marrow infiltration and light chain type MM. The 5-year disease-specific survival was 82% and 30% in patients with sFLCR lower than and equal or greater than the median, respectively ( P = 0·0001). sFLCR was an independent prognostic factor. [ABSTRACT FROM AUTHOR]

Summary. Bone marrow angiogenesis has recently been implicated in the pathophysiology and course of various haematological malignancies. Little is known, however, about the significance of this phenomenon in hairy cell leukaemia (HCL). We evaluated various morphometric characteristics of microvessels, highlighted by means of anti-CD34 immunohistochemistry, in the bone marrow of 44 patients with typical HCL, before and after treatment with interferon-α (IFN-α). Overall, bone marrow from 103 HCL patients and 20 controls was examined. Microvessel density (MVD) and several size- and shape-related parameters were quantified in the region of most intense vascularization using image analysis. MVD, size-related parameters and the percentage of branching microvessels were higher in HCL than in controls. Likewise, perimeter counts were higher in partial/non-responders than in complete responders. Achievement of complete response was accompanied by smaller calibre microvessels. IFN-α induced a decrease in MVD and branching values in cases with diffuse marrow involvement. In univariate analysis, progression-free survival was adversely affected by MVD, branching and major axis length. Multivariate analysis indicated that MVD/branching independently affected progression-free survival and the likelihood of complete response. Our data suggest that the generation of bone marrow microvessels indicated an increased risk of progression and IFN-α treatment failure in HCL. Furthermore, the prognostic significance of angiogenesis requires the concomitant assessment of MVD and the complexity of the microvascular network. [ABSTRACT FROM AUTHOR]

The standard CHOP regimen may cure 30-40% of patients with advanced aggressive non-Hodgkin's lymphoma (ANHL). Mitoxantrone is an anthracenedione, which is active in NHL and its toxicity profile may be more favorable than doxorubicin with respect to alopecia, mucositis and cardiotoxicity. This study was designed to compare the effectiveness of an escalated dose of mitoxantrone with that of standard doxorubicin, used in the CHOP regimen in patients with ANHL. One hundred and forty three eligible patients with ANHL were randomized to receive 6 cycles of either CHOP (n = 71) or intensified CNOP (iCNOP) (n = 72), with mitoxantrone 20 mg/m², i.v., d.1 instead of doxorubicin. Complete responders (CR) were again randomized either to receive interferon-α (IFN-α) maintenance (3 MU t.i.w., s.c.) or not. The CR rate was 70 vs. 76% for iCNOP and CHOP (p = 0.45), and the overall response rate was 81 vs. 83%, respectively (p = 0.71). The 5-year failure free survival (FFS) was 48 and 50% in the iCNOP and CHOP arm, respectively (p = 0.45), and the 5-year overall survival (OS) was 61 vs. 64% (p = 0.56). IFN-α did not prolong relapse free survival (p = 0.91). iCNOP produced less alopecia (p = 0.001) but more febrile episodes (p = 0.04) than CHOP, while requiring more frequent G-CSF support (p = 0.01). Two cases of acute myelogenous leukemia (AML) were recorded, both in the iCNOP arm (p = 0.14). In conclusion, iCNOP was equally effective to CHOP in patients with ANHL, producing more leukopenia and febrile episodes, but less alopecia. The development of two cases of secondary AML in the iCNOP arm is of concern. [ABSTRACT FROM AUTHOR]

Pulmonary involvement in Waldenström’s macroglobulinaemia (WM) occurs in 3–5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.Copyright © 2001 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]