BAL GM is a valuable adjunctive diagnostic tool to other conventional microbiologic and radiologic studies.
Introduction: Mantle cell lymphoma is a B-cell non-Hodgkin's lymphoma characterized by a t (11;14), resulting in overexpression of cyclin D1. Conventional chemotherapy obtains frequent (but short) remissions, leading to a poor median overall survival (OS) of 3-5 years. To obtain more information about the prevalence and current treatment of Mantle cell lymphoma (MCL) in Belgium, we collected data in a Belgian registry of MCL. Materials and methods: All Belgian MCL patients, t(11;14) and/or cyclin D1 positive, seen in hematology departments over a one-year period (April 2013-March 2014) were included. Data about patient characteristics, histology, treatment lines, and response were compiled and retrospectively analyzed. Results: Four hundred and four patients were included with a median age at diagnosis of 64 years (range 23-96 years) and a male predominance (72%). For 2013, we calculated a prevalence of at least 36.2 per million and an incidence of at least 7.0 per million in the Belgian population. Characteristics at diagnosis involved lymphadenopathy (82%), splenomegaly (44%), B-symptoms (39%), and hepatomegaly (10%). Bone marrow invasion was present at diagnosis in 77%. Stage at diagnosis was advanced in the majority of cases. The median number of treatment lines was 1. Type of first line treatment included a combination of anthracyclin and cytarabinebased regimen (34%), anthracyclin (39%), and other. Rituximab was used in 88% of first line treatments. In 44% first line treatment was followed by autologous stem cell transplantation. Conclusion: The analysis of this Belgian MCL registry provides insight in the epidemiology, demographics, and current treatment of our Belgian MCL population.
We report an unusual case of systemic anaplastic large cell lymphoma (ALCL), ALK positive with leukemic involvement in a 57-year-old woman. The patient presented with a fulminant respiratory infection unresponsive to treatment requiring intensive care and ventilatory support. The CT scan demonstrated mediastinal and bilateral lymphadenopathy. On peripheral smear a few atypical lymphocytes were visualized. Based on the increasing number of atypical lymphocytes in the daily peripheral bloodsmears, the diagnosis ALCL was suggested. Definitive diagnosis was made on a bone marrow biopsy, with lymphocytes being immunoreactive for CD30, EMA, and ALK. Leukemic peripheral blood involvement in ALCL is an uncommon clinicopathologic entity with unfavorable prognosis. The case we present is perhaps unusual in that a complete respons was achieved, highlighting the importance of prompt diagnosis and judicious management.
Background:Since 2009 treatment of chronic ITP patients with thrombopoietin receptor agonists (TPO‐RA) (romiplostim‐eltrombopag) has been reimbursed in Belgium. Despite the initial assumption that this would be a life‐long therapy, practical experience has shown that a significant number of patients do stop treatment. Reasons of TPO‐RA discontinuation can be no efficacy, adverse events, ITP or non‐ITP related death or spontaneous remission. Spontaneous remission (SR) or treatment free‐remission (TFR) is defined as a treatment‐free period of 3 6 months (mo) with platelets 3 50000/ml and no need of ITP rescue medication.Aims:We performed a survey to know how many patients were treated with TPO‐RA (trial, reimbursed, medical need) and how many of these had stopped this treatment. More details were gathered of patients who discontinued treatment.Methods:The survey was sent by mail to all hematologists who were member of the Belgian Hematological Society.Results:20 Belgian centers participated in our survey and sent data. 42 patients fulfilled the definition of TFR, while 3 of these patients had several episodes of TFR. The baseline characteristics of the patients obtaining TFR were as follows: 26 patients were male and 16 were female; diagnosis of primary ITP was made in 34 patients; the 8 patients with secondary ITP had an underlying lymphoma (n = 5), an Evans syndrome (n = 2) or a previous transplant (n = 1); mean age at ITP diagnosis was 50.5 years (y) (range 1–87y); median platelet count at ITP diagnosis was 5000/ml; median time between ITP diagnosis and the start of TPO‐RA was 5 y (range 1 mo‐24y); at start of TPO‐RA 5 patients fulfilled the definition of acute, 8 of persistent and 28 of chronic ITP; 54% (22/41) were splenectomized; 55% (22/40) needed 3 3 ITP treatments before the start of the TPO‐RA (corticosteroids, intravenous immunoglobulins, rituximab, cyclophosphamide, cyclosporin, fansidar…); median platelet count at start of TPO‐RA was 11000/ml; median time on TPO‐RA was 8 mo (range 0–74 mo); median platelet count was 267000/ml at discontinuation of TPO‐RA and 177000/ml 6 mo later. 178 patients (provided by 15 centers) were treated with TPO‐RA. Of these 100 patients discontinued treatment. 21% of the total cohort discontinued TPO‐RA because of TFR and 37% of the discontinuations was due to TFR.Summary/Conclusion:Our survey confirmed that SR or TFR on TPO‐RA is seen in about 20% of ITP patients treated in daily clinical practice in Belgium. TFR is seen not only in acute or persistent ITP but also in chronic ITP. TFR is seen in patients independent of splenectomy and independent of the number of treatments given before. In the future the incidence of TFR is thought to be even higher when the use of a forced tapering schedule will become common practice.
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