Front-line venetoclax monotherapy for blastic plasmacytoid dendritric cell neoplasm

BJH - volume 15, issue 3, may 2024

C. Maquet MD, B. De Prijck MD, A. Jaspers MD, PhD, F. Tassin MD, PhD, A. Keutgens MD, PhD, Y. Beguin MD, PhD, A. De Voeght MD


Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive haematological disease with a grim prognosis. The majority of patients at diagnosis are elderly (typical age range: 60 to 70 years) and seldom fit enough to receive intensive induction therapy. We report here the case of a 76-year-old patient with significant cardiac comorbidity at the time of the diagnosis of BPDCN with bone marrow involvement. He achieved haematologic complete remission with venetoclax as first-line therapy with preservation of a good quality of life, an almost exclusively outpatient care and transfusion independency. The only adverse event was a grade 4 neutropenia without any infectious complication.

(BELG J HEMATOL 2024;15(3):125–9)

Read more

Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS syndrome) with multiple thromboembolism events: A Belgian case report

BJH - volume 14, issue 8, december 2023

M. Pirotte MD, A. De Voeght MD, G. Vertenoeil MD, PhD, M. Vasbien MD, H. Paridaens MD, J. Somja MD, PhD, P. Collins MD, F. Lambert MD


VEXAS syndrome, an acquired autoinflammatory syndrome, is classified within the complex of autoinflammatory diseases (AID), arising from aberrant changes in the innate immune system due to acquisition of somatic mutation of the UBA1 gene in bone marrow cells. This recently identified syndrome is characterised by systemic inflammation, chondritis, neutrophilic dermatosis, pulmonary involvement, thrombosis, macrocytosis and cytopenia in mature men. We present a case study of a 67-years-old man exhibiting multiple thrombotic manifestations without any known underlying aetiology or haemopathy. This report emphasises the crucial collaboration between clinicians, cytologists and geneticists highlighting the pivotal role of UBA1 mutation screening in the diagnostic process to confirm the final diagnosis.

(BELG J HEMATOL 2023;14(8):336–42)

Read more

Magrolimab: A new weapon against cancer cells

BJH - volume 14, issue 6, october 2023

A. De Voeght MD


Magrolimab is a first-in-class monoclonal antibody that targets and inhibits CD47, a “don’t eat me” signal overexpressed on the surface of cancer cells that allows them to evade the innate immune response. Blocking CD47 leads to increased recognition and phagocytosis of cancer cells. Since its initial description and promising data in pre-clinical and clinical studies, three phase III studies investigating magrolimab in AML patients are ongoing in Belgium and worldwide.

(BELG J HEMATOL 2023;14(6):250–4)

Read more

Venetoclax-Azacitidine, the new standard of care for AML patients unfit for intensive treatment: A guide for clinical practice

BJH - volume 14, issue 2, march 2023

I. Moors MD, D. Deeren MD, C. Jacquy MD, PhD, A. Jaspers MD, PhD, T. Kerre MD, PhD, V. Havelange MD, PhD, D. Selleslag MD, C. Spilleboudt MD, N. Straetmans MD, PhD, F. Van Obbergh MD, A. De Voeght MD, S. Anguille MD, PhD, A. Schauwvlieghe MD, PhD, N. De Beule MD, PhD, A. De Becker MD, D. Breems MD, PhD


Acute myeloid leukaemia is an aggressive form of bone marrow cancer with poor prognosis, especially in elderly, unfit patients. The VIALE-A study showed an impressive improvement in complete remission rate and overall survival with the addition of venetoclax, a BCL-2 inhibitor, to azacitidine. This combination therapy is now reimbursed in Belgium for newly diagnosed adult AML patients who are considered unfit for intensive chemotherapy based on age and/or comorbidities. In this article, we provide recommendations on the use of this new combination, as well as on prophylaxis and management of specific side effects.

(BELG J HEMATOL 2023;14(2):59–66)

Read more