BJH - volume 7, issue 3, june 2016
C. Springael MD, PhD, V. Delrieu MD, K.L. Wu MD, PhD, W. Schroyens MD, PhD, C. Bonnet MD, PhD, D. Bron MD, PhD, A. Janssens MD, PhD, On behalf of the BHS Lymphoproliferative Working Party
Large granular lymphocyte and prolymphocytic leukaemias are rare chronic lymphoproliferative disorders. Large granular lymphocyte leukaemias consist of indolent disorders such as T-cell large granular lymphocyte and chronic lymphoproliferative disorder of natural killer cells and the very rare but aggressive natural killer cell leukaemia. Treatment of the indolent large granular lymphocyte leukaemias is necessary in case of symptomatic cytopaenias or non-haematological autoimmune disorders. First line therapy of these two disorders is based on three immunosuppressive drugs: methotrexate, cyclophosphamide and cyclosporine A. Aggressive natural killer cell leukaemia needs an L-asparaginase containing regimen as induction followed by allogeneic stem cell transplantation to prolong remission. T-cell prolymphocytic leukaemia always follows an aggressive course even after an indolent onset. The optimal treatment strategy should exist of remission induction with alemtuzumab intravenously followed by autologous or allogeneic stem cell transplantation. Treatment indications for B-cell prolymphocytic leukaemia follow the criteria described by the chronic lymphocytic leukaemia guidelines. After induction with fludarabine, cyclophosphamide, rituximab or bendamustine in patients without a p53 mutation and/or a 17p deletion and alemtuzumab in case of a p53 mutation and/or a 17p deletion, stem cell transplantation must be considered.
(BELG J HEMATOL 2016; 7(3):103–11)
Read moreBJH - volume 7, issue 2, april 2016
V. De Wilde MD, PhD, D. Dierickx MD, PhD, W. Schroyens MD, PhD, E. Van den Neste MD, PhD, C. Bonnet MD, PhD, M. André MD, A. Janssens MD, PhD, V. Van Hende MD, A. Van Hoof MD, PhD
Primary central nervous system lymphoma is a rare form of extranodal B cell lymphoma of the brain, the eyes, the meninges or the spinal cord in the absence of systemic lymphoma. The management of primary central nervous system lymphoma remains controversial, which is related to the rarity of the cases and the small number of controlled studies available. The present consensus report provides the guidelines proposed by the Belgian Hematology Society Lymphoproliferative Working Party for treating immunocompetent adult patients with primary central nervous system lymphoma.
(BELG J HEMATOL 2016;7(2):69–78)
Read moreBJH - volume 6, issue 4, october 2015
V. Van Hende MD, D. Bron MD, PhD, E. Van den Neste MD, PhD, C. Bonnet MD, PhD, M. André MD, A. Van Hoof MD, PhD, D. Dierickx MD, PhD, G. Verhoef MD, PhD, T. Tousseyn MD, PhD, A. Janssens MD, PhD, V. De Wilde MD, PhD, K.L. Wu MD, PhD, P. Heimann MD, PhD
Waldenström’s macroglobulinaemia is a B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with demonstration of an IgM monoclonal gammopathy in the blood. This condition belongs to the lymphoplasmacytic lymphomas as defined by the World Health Organization classification (ICD-0 code 9671/3). Approximately one-fourth of patients are asymptomatic. Clinical features of the symptomatic patients are diverse and may relate to overall disease burden (such as peripheral blood cytopaenias, organomegaly and constitutional symptoms) or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome, amyloidosis, peripheral neuropathy and cold haemagglutinin. Therapeutic options have traditionally involved alkylating agents, nucleoside analogues, and rituximab, either as single therapy or in combination. However, emerging new data on combination therapy as well as novel agents have shown encouraging results. This report provides the Belgian Hematology Society guidelines according to recent clinical studies.
(BELG J HEMATOL 2015;6(4):142–50)
Read moreBJH - volume 6, issue 4, october 2015
C. Bonnet MD, PhD, A. Bosly MD, PhD
17 – 20 June 2015, Lugano, Switzerland
A lot of interesting data were presented at the 13th International Conference on Malignant Lymphoma in Lugano, Switzerland. The authors summarise below those presentations/abstracts they found relevant for daily practice, either now or in the near future.
(BELG J HEMATOL 2015;6(4): 173–8)
Read moreBJH - volume 6, issue 2, may 2015
C. Bonnet MD, PhD, A. Janssens MD, PhD, K.L. Wu MD, PhD, W. Schroyens MD, PhD, V. Van Hende MD, P. Heimann MD, PhD, T. Tousseyn MD, PhD, M. André MD, D. Bron MD, PhD, A. Van Hoof MD, PhD, G. Verhoef MD, PhD, B. De Prijck MD, Y. Beguin MD, PhD, D. Dierickx MD, PhD
Burkitt’s lymphoma is a rare but very aggressive non-Hodgkin’s lymphoma characterised by an isolated translocation t(8;14)(q24;q32). The sporadic form is the sub-entity most frequently encountered in Belgium. Diagnosis and initial work-up must be completed rapidly to start treatment as soon as possible. Positron emission tomography scan is useful for initial staging and to evaluate the chemosensitivity of the tumour during and after treatment. After debulking, it is recommended to add rituximab to chemotherapy. Currently intensive short-cycle and low intensity chemotherapies are two valuable options. Radiotherapy is not indicated except in case of central nervous system involvement. Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured. For patients in partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies.
(BELG J HEMATOL 2015;6(2):61–9)
Read moreBJH - volume 6, issue 2, may 2015
S. Sid MD, C. Dugauquier MD, B. De Prijck MD, C. Bonnet MD, PhD, Y. Beguin MD, PhD
We present a patient with Burkitt’s lymphoma who suffered a severe haemolytic crisis after treatment with rasburicase. This case report underlines the high incidence of glucose-6-phosphate dehydrogenase deficiency in some ethnic groups and the importance of a detailed patient and family history before starting treatment, even in case of emergency. Glucose-6-phosphate dehydrogenase is an essential enzyme since it makes the synthesis of NADPH + H from NADP possible, which determines the reducing power (NADPH) of the cell. Every defect in this physiological process, notably glucose-6-phosphate dehydrogenase deficiency, may thus result simultaneously with the use of rasburicase in acute or chronic haemolysis according to the importance of the deficiency. Management is based on stopping the incriminated drug and on supportive therapy consisting of administering packed red blood cells if the anaemia is poorly tolerated.
(BELG J HEMATOL 2015;6(2): 74–8)
Read moreBJH - volume 6, issue Abstract Book BHS, january 2015
J. Foguenne , M. Simul , R. Keutgens , F. Tassin , C. Bonnet MD, PhD, Y. Beguin MD, PhD, M. Jamar , F. Lambert MD, A. Gothot MD, PhD
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