D. Bron MD, PhD

Adult T-cell leukaemia-lymphoma: Pathogeny and clinical recommendations

SUMMARY

Human T-cell leukaemia virus type 1 (HTLV-1) was the first human oncogenic virus discovered. It is endemic in some regions of the world but increasingly prevalent in our countries as globalisation is progressing. After several decades of asymptomatic carrying, approximately 2–5% of infected individuals will develop adult-T-cell leukaemia-lymphoma (ATL). Despite significant progress in the physiopathology and therapeutic interventions, the prognosis of this rare disease is dismal. An update of classification, clinical features, diagnosis and recent treatment recommendations is outlined in this review.

(BELG J HEMATOL 2019;10(7):277–84)

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)

EHA Scientific Working Group meeting on ageing and haematology

After a successful first edition in Lisbon, a second edition of this scientific working group meeting was held in Warsaw in October 2018. The objective was to organise roundtables with scientists, clinicians, onconurses and patient’s organisations to improve the management of older patients with haematological disorders. Several unsolved issues were debated as outlined below.

(BELG J HEMATOL 2019;10(3):127–9)

Clinical and biologic correlates of frailty in older patients with malignant hemopathies

Frailty assessment in older patients with malignant hemopathies is very useful in order to improve care and treatment options. However, some lacks of data exist regarding the unsuspected frail population in presumed ‘clinically fit’ patients who should not benefit from chemotherapy. In this article, we review current data regarding prognostic factors and frailty scoring in older patients with malignant hemopathies. Prospective trials are needed to build a new frailty scoring to assess the unsuspected frail population in ‘clinically fit’ patients including specifically assessment of cognitive impairment.

(BELG J HEMATOL 2019;10(2):65–8)

Management of cutaneous T & B cell lymphomas: a comprehensive review

SUMMARY

Primary cutaneous lymphomas are a heterogeneous group of diseases with indolent or aggressive behaviour, skin-limited or systemic extension, from T or B cell origin. The optimal management requires the multi-disciplinary approach with dermatologists, hemato-oncologists, pathologists and molecular biologists. The objective of this review is to harmonise the work-up and the treatment of these different entities of cutaneous T or B cell lymphoma in Belgium, according to the availability of the drugs and specialised treatment such as extracorporeal photopherisis or total skin electron beam therapy.

(BELG J HEMATOL 2018;9(3):86–100)

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)

PP55 Transcription factors and checkpoint inhibitors expression in t-lymphocytes from chronic lymphocytic leukemia patients and age-matched healthy donors