A. Van Hoof MD, PhD

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)

Updated BHS guidelines for the treatment of chronic lymphocytic leukaemia, mantle cell lymphoma and Waldenström macroglobulinemia anno 2018

SUMMARY

The Belgian Haematological Society Lymphoproliferative Working Party updated the existing recommendations on best strategies for frontline and subsequent line treatment of small lymphocytic leukaemia/chronic lymphocytic leukaemia, mantle cell lymphoma and Waldenström Macroglobulinemia according to new reimbursements and robust clinical data.

(BELG J HEMATOL 2018;9(3):101–12)

P15 Treatment patterns of Waldenström’s macroglobulinemia in Belgium

BHS guidelines for primary central nervous system lymphoma

Summary

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)

Highlights in aggressive lymphomas

(BELG J HEMATOL 2016; 7(1): 42–4)

Treatment of mantle cell lymphomas: Updated recommendations of the Belgian Hematological Society 2015

summary

Mantle cell lymphoma is a rare B-cell non-Hodgkin’s lymphoma characterised by a t(11;14) translocation resulting in overexpression of cyclin D1 and cell cycle dysregulation. Mantle cell lymphoma represents approximately 7–9% of all lymphomas in Europe.¹ Although new treatment regimens have improved the outcomes over the last decades, mantle cell lymphoma is still considered one of the worst prognosis B-cell non-Hodgkin’s lymphoma with a median overall survival of less than five years.² In September 2014 the Belgian Hematological Society recommendations for the treatment of mantle cell lymphoma were published.³ Since then, novel therapies such as ibrutinib and bortezomib have been approved by the European Medicines Agency in the treatment of mantle cell lymphoma. We present the new updated recommendations of the Belgian Hematological Society Lymphoproliferative Working Party. For young patients, the first line therapy remains an AraC-containing chemo-immunotherapy followed by high dose chemotherapy and autologous stem cell transplantation. For the main group of elderly patients, chemo-immunotherapy followed by maintenance with rituximab appears to be the gold standard. In relapse we can recommend treatment with BTK-inhibitor ibrutinib as first choice. Temsirolimus is reimbursed as third line treatment. Relapse patients should also be considered for allogeneic stem cell transplantation if eligible.

(BELG J HEMATOL 2015;6(5):203–8)

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

summary

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)