Articles

Highlights in chronic lymphocytic leukaemia

BJH - volume 11, issue 5, september 2020

A. Janssens MD, PhD

SUMMARY

We refer to the recent Belgian Haematological Society (BHS) guidelines for the management of small lymphocytic lymphoma (SLL) and chronic lymphocytic leukaemia (CLL) made by the BHS lymphoproliferative disease committee. The 2020 publication is already the third update after the initial paper published in 2012. For diagnosing CLL, we recommend using the immunophenotype panel according the European research initiative on CLL and European society for clinical cell analysis (ERIC/ESCCA) harmonization project. Concerning diagnostic and pre-treatment work-up, we advise testing of the IGVH mutational status only in young ‘and’ older patients without a 17p del/TP53 mutation if chemo-immunotherapy (CIT) could be a therapeutic option. We are waiting for reimbursement of the IGVH testing in this small number of elderly patients. The recently updated international workshop on CLL (iwCLL) guidelines added as indication for initiating treatment ‘symptomatic functional extranodal disease’. Although criteria for initiating first-line or second-line treatment follow in general similar rules, the same iwCLL guidelines stress that subsequent therapy can be acceptable, sometimes in overlap with the previous one, in patients when substantial disease persists or disease progresses under novel agents, even if the patient stays asymptomatic, to avoid Richter-like acceleration.1 At EHA 2020, an update on treatment advances in previously untreated and relapsed/refractory CLL was given. This overview will provide an update on the most important study results and how these results might change the current guidelines in the treatment of CLL.

(BELG J HEMATOL 2020;11(5):209-15)

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Ibrutinib and bleeding management: a Belgian expert consensus

BJH - volume 11, issue 4, june 2020

A. Janssens MD, PhD, D. Bron MD, PhD, V. Van Hende MD, V. Galle MD, K. Jochmans MD, PhD, S. Meers MD, PhD, M. André MD, PhD, M-C. Ngirabacu MD, PhD, K.L. Wu MD, PhD, B. De Prijck MD, P. Verhamme MD, PhD, C. Hermans MD, PhD

SUMMARY

In recent years ibrutinib emerged as a paradigm shifting agent in the treatment of chronic lymphocytic leukaemia (CLL), mantle cell lymphoma (MCL) and Waldenström’s macroglobulinemia (WM). In clinical trials and in real-world studies ibrutinib proved to be an effective agent with an overall favourable tolerability profile. However, compared with standard chemo-immunotherapy (CIT), ibrutinib was associated with a higher incidence of clinically significant bleeding. This has been hypothesized to be linked to the platelet-specific effects of inhibiting Bruton’s tyrosine kinase (BTK). Most bleeding events under ibrutinib are low-grade with a decreasing incidence over time. However, bleeding can have a significant impact on patients and interfere with persistence and compliance of ibrutinib treatment. Currently, no clear consensus exists on the use of ibrutinib in patients with an increased bleeding risk, on the management of ibrutinib-induced bleeding and on the use of ibrutinib around surgery or invasive procedures. In this paper, a panel of Belgian haematology and haemostasis specialists formulated practical advice on bleeding prevention and management in ibrutinib-treated patients.

(BELG J HEMATOL 2020;11(4):174–84)

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BHS Guidelines for the Management of Small Lymphocytic Lymphoma and Chronic Lymphocytic Leukaemia, anno 2020

BJH - volume 11, issue 3, may 2020

A. Janssens MD, PhD

SUMMARY

The Belgian Haematological Society (BHS) Lymphoproliferative Disease Committee updated the existing recommendations on diagnosis, prognostic scores, treatment indications, best strategies for front-line and subsequent-line treatment of small lymphocytic lymphoma (SLL)/ chronic lymphocytic leukaemia (CLL), according robust new data.

(BELG J HEMATOL 2020;11(3):108–19)

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Guidelines of the Belgian Hematological Society for newly diagnosed and relapsed follicular lymphoma anno 2019

BJH - volume 11, issue 2, march 2020

M. Clauwaert MD, V. Galle MD, M. Maerevoet MD, A. Janssens MD, PhD, K. Saevels MD, S. Snauwaert MD, PhD, C. Springael MD, PhD, V. Van Hende MD, G. Verhoef MD, PhD, F. Offner MD, PhD

SUMMARY

Follicular lymphoma is the most common low-grade non-Hodgkin lymphoma. Survival rates have been rising over time mainly due to advancing therapeutic strategies. As the last Belgian guidelines date from 2012, we present an update of the scientific evidence regarding diagnosis, staging, treatment and follow-up, and confront these to the Belgian reimbursement rules anno 2019. Follicular lymphoma grade 3B is classified as high-grade lymphoma and treated accordingly, and will not be discussed in this paper. Early stage disease can be treated with involved-field radiotherapy, which has curative potential. Advanced stage disease is virtually incurable, but many treatment options are available with good results. In first line, treatment is mostly based on chemotherapy combined with rituximab; the latter can be continued as maintenance therapy. In relapsed setting, introduction of the newer and more potent anti-CD20-antibody obinutuzumab, also in combination with chemotherapy, can lead to improved survival in high-risk patients. For older patients with comorbidities, rituximab monotherapy is the preferred option. In further lines, PI3K-inhibition with idelalisib and radioimmunotherapy are available. Finally, autologous or allogeneic stem cell transplantation remain an option in a small group of selected patients.

(BELG J HEMATOL 2020;11(2):67–74)

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BHS guidelines for the treatment of newly diagnosed diffuse large B-cell lymphoma (DLBCL) anno 2020

BJH - volume 11, issue 2, march 2020

G. Swennen MD, A. Janssens MD, PhD, V. Vergote MD, S. Bailly MD, C. Bonnet MD, E. Van den Neste MD, PhD, M. Maerevoet MD, S. Snauwaert MD, PhD, K. Saevels MD, C. Jacquy MD, PhD

SUMMARY

Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma. Prognosis of diffuse large B-cell lymphoma has improved dramatically since the introduction of rituximab and about two thirds of patients can be cured with immunochemotherapy. In the last twenty years, it became clear that diffuse large B-cell lymphoma is a very heterogeneous disease and based on the genetic mutation landscapes numerous efforts have been made to develop novel treatment strategies to improve the prognosis of diffuse large B-cell lymphoma further. This article provides an update of diagnosis, current treatment guidelines and novel treatment strategies for newly diagnosed patients with diffuse large B-cell lymphoma in Belgium. It will also focus on treatment of elderly patients and high-grade B-cell lymphoma.

(BELG J HEMATOL 2020;11(2):56–66)

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Highlights in chronic lymphocytic leukaemia

BJH - volume 10, issue 5, september 2019

A. Janssens MD, PhD

SUMMARY

During the last years several phase III studies comparing targeted agents (alone or in combination) to conventional chemo immunotherapy (CIT) have been published in chronic lymphocytic leukaemia (CLL) and small lymphocytic leukaemia (SLL). EHA 2019 featured several presentations on the most recent progress in this field. A selection of abstracts, as well as their impact on clinical practice, are discussed below.

(BELG J HEMATOL 2019;10(5):214–9)

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

BJH - volume 10, issue 4, june 2019

D. Bron MD, PhD, M. Maerevoet MD, E. Van den Neste MD, PhD, V. Delrieu MD, F. Offner MD, PhD, W. Schroyens MD, PhD, A. Van Hoof MD, PhD, G. Verhoef MD, PhD, J.B. Giot MD, J.P. Loly MD, A. Janssens MD, PhD, C. Bonnet MD

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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