BJH - volume 14, issue 1, february 2023
B. Sciot MD
This article will give an overview of the key studies in chronic lymphocytic leukaemia (CLL) and indolent non-Hodgkin lymphoma (iNHL) that were presented at the 2022 annual meeting of the American Society of Hematology. In CLL, topics mainly focused on the combination of novel agents and there are new data from head-to-head comparison of different Bruton’s Tyrosine Kinase inhibitors (BTKi). In follicular lymphoma, bispecific T-cell engagers continue to make their introduction in the therapeutic landscape, whereas CAR-T cells are showing durable responses.
(BELG J HEMATOL 2023;14(1):16–8)Read more
BJH - volume 13, issue 2, march 2022
B. Sciot MD, T. Devos MD, PhD, T. Tousseyn MD, PhD, N. Boeckx MD, PhD, L. Michaux MD, PhD, P. Vandenberghe MD, PhD
Introduction: Advanced systemic mastocytosis is a rare myeloproliferative disorder of mast cells, damaging the function of various organs and tissues. The diagnosis can be challenging due to its protean manifestations and rareness. Treatment options have improved over the last years. Currently, avapritinib, a novel tyrosine kinase inhibitor with activity against p.D816V mutated KIT, is under investigation.
Case: We report a case of a 64-year old man with chronic diarrhoea, fatigue, weight loss and ascites with hepatomegaly, developing an upper gastro-intestinal bleeding with multiple duodenal ulcers. Diagnostic work-up revealed hepatosplenomegaly and portal hypertension, a vertebral compression fracture and multiple 18F-FDG avid supra- and infradiaphragmatic lymph nodes and bone marrow. Based on the 2016 WHO criteria of systemic mastocytosis, and a concomitant chronic myelomonocytic leukaemia, the diagnosis of an aggressive systemic mastocytosis with an associated haematological neoplasm was made. The patient was consecutively treated with midostaurin, cladribine and avapritinib, the latter inducing a complete biochemical and molecular response.
Conclusion: This case illustrates the challenging clinical presentation of systemic mastocytosis. A deep response to avapritinib was observed despite prior use of midostaurin and cladribine, underlining its promise in advanced systemic mastocytosis.
(BELG J HEMATOL 2022;13(2):84–91)Read more