V. Delrieu MD

BHS guidelines for the treatment of marginal zone lymphomas: 2018 update

Marginal zone lymphomas (MZL) are a heterogeneous subtype of indolent B-non-Hodgkin lymphomas that includes distinct entities:

• Extranodal mucosa-associated lymphoid tissue lymphoma arises in a variety of tissue but primarily in the stomach. They are usually localised and often associated with chronic antigenic stimulation by microbial pathogens. Eradication of the pathogen is a major part of the first-line therapy. The prognosis is excellent in early stages. In advanced stages, observation, anti-CD20 antibodies and/or cytostatic drugs are therapeutical approaches.
• Nodal MZL is usually confined in lymph nodes, bone marrow and peripheral blood. The prognosis is somewhat worse in this entity. Current recommendations suggest that they should be managed as follicular lymphomas.
• Splenic MZL is a unique entity involving the spleen, bone marrow and blood. Hepatitis infection should be eradicated before considering treatment. These lymphomas have an indolent behaviour, and only symptomatic patients should be treated by splenectomy and/or anti-CD20 antibodies.
• Two novel entities are described, non-chronic lymphocytic leukaemia monoclonal B-cell lymphocytosis, probably closely related to splenic MZL lymphoma, and a less well-defined provisional entity involving primarily the spleen called splenic B-cell lymphoma/leukaemia, unclassifiable, including splenic diffuse red pulp lymphoma and hairy-cell leukaemia variant.

This review will discuss separately the diagnosis, work-up and treatment of extranodal mucosa-associated lymphoid tissue lymphoma, nodal MZL and splenic MZL. These guidelines include the recently published ESMO consensus conference on malignant lymphoma.^1–3

(BELG J HEMATOL 2019;10(4):153–64)

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BHS guidelines for the diagnosis and the treatment of hairy cell leukaemia

SUMMARY

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)

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BHS guidelines for the treatment of large granular lymphocyte and chronic prolymphocytic leukaemias

Summary

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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Update on the initial therapy of multiple myeloma

Summary

With the introduction of immunomodulatory drugs and proteasome inhibitors, major improvements have been achieved in the treatment and prognosis of multiple myeloma. Different treatment combinations are now in use and innovative therapies are being developed. This rapidly changing therapeutic landscape calls for an update on the Belgian myeloma guidelines, published in 2010.¹ Based on an extensive review of the recent literature, the myeloma study group of the Belgian Hematology Society has revised the consensus recommendations on myeloma care, to be used by haematologists as a reference for daily practice. When applicable, comments with regards to the Belgian reimbursement modalities are included. The full text with appendices can be downloaded from the Belgian Hematology Society website (www.bhs.be) and from the Belgium Journal of Hematology website (www.ariez.com).

(BELG J HEMATOL 2014;5(4):125–36)

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