BJH - volume 8, issue 6, october 2017
V. Delrieu MD, C. Springael MD, PhD, K.L. Wu MD, PhD, G. Verhoef MD, PhD, A. Janssens MD, PhD, On behalf of the BHS Lymphoproliferative Working Party
Hairy cell leukaemia is a rare chronic B-cell lymphoproliferative disorder characterised by a long natural course with, in most of cases, an excellent response to a single course of purine analogue monochemotherapy. Making the right diagnosis, excluding the chemo resistant variant form of hairy cell leukaemia, and making progresses in the treatment of relapsing and/or refractory disease remains challenging up to date. In recent years, exciting results with new agents are emerging and clinical trials are ongoing to optimize the management of hairy cell leukaemia and its variant form.
(BELG J HEMATOL 2017;8(6):222–8)
Read moreBJH - 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, 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)
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