PRACTICE GUIDELINES

Ibrutinib and bleeding management: a Belgian expert consensus

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)

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

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)

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

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)

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

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)

Diagnostic testing in myeloid malignancies by next-generation sequencing: recommendations from the Commission Personalised Medicine

SUMMARY

Molecular diagnostics have an increasing impact on diagnosis, risk stratification and targeted treatment in haemato-oncology. In the framework of a pilot study for the implementation of next-generation sequencing in the Belgian healthcare system, the Commission of Personalised Medicine was founded to give professional and evidence-based advice on the molecular analysis in haemato-oncology. This paper describes its recommendations for NGS analysis in myeloid malignancies. In addition, the minimally required set of genes that must be analysed is defined and algorithms for molecular workflow in myeloid malignancies are proposed.

(BELG J HEMATOL 2019;10(6):241–9)

BHS clinical guidelines on the management of acute complications in sickle cell disease

With the increasing prevalence of sickle cell disease patients in Western countries, it is of importance to improve awareness among medical doctors of its complications. To reduce long-term morbidity and mortality, the prompt recognition and treatment of acute complications is important. The existing clinical guideline ‘Follow-up and treatment of patients with sickle cell disease hospitalised for Vaso Occlusive Crisis or infection’, published in 2012 by the Belgian Haematological Society, was revisited to better suit the practical needs of first-line practitioners.

(BELG J HEMATOL 2019;10(4):165–8)

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)