BJH - volume 11, issue Abstract Book BHS, february 2020
T. Devos MD, PhD, J. Gotlib , E. Asatiani , C. Walker , H. Zhen , S. Verstovsek
BJH - volume 10, issue 7, november 2019
T. Goos , G. Verhoef MD, PhD, T. Devos MD, PhD
Systemic mastocytosis is a rare heterogeneous disorder characterised by abnormal proliferation of mast cells. It can be divided in subtypes with different phenotypes and prognoses. This article is a retrospective descriptive study of 37 patients with systemic mastocytosis according to the WHO criteria of 2008 at UZ Leuven. Twenty-eight patients had indolent systemic mastocytosis (75.7%), four smouldering systemic mastocytosis (10.8%), three aggressive systemic mastocytosis (8.1%) and two systemic mastocytosis with associated haematological neoplasia (5.4%). In one out of five patients, the diagnosis was made based on at least three minor criteria. The sensitivity of CD25 expression was 100%. In 75.7% a KITD816V mutation was detected. The value of serum tryptase was associated with the subtype of systemic mastocytosis. Seventy-eight percent had cutaneous lesions. Forty-eight percent experienced at least one anaphylactic reaction. Osteoporosis was reported in 24.6%. Eighty-nine percent and 64.9% of patients were treated with respectively H1 and H2 antihistamines. Cytoreductive treatment was administered to nine patients (24.3%). Three patients received as first-line treatment midostaurine, three imatinib, one cladribine, one nilotinib and one masitinib. This study reflects the heterogeneity of this condition and emphasises the importance of a multidisciplinary approach in a specialised center for early diagnosis and treatment of disease-associated manifestations.
(BELG J HEMATOL 2019;10(7):265–76)
Read moreBJH - volume 9, issue 3, june 2018
Y. Serroukh MD, PhD, H. Claerhout MD, A. Janssens MD, PhD, T. Tousseyn MD, PhD, N. Boeckx MD, PhD, J. Maertens MD, PhD, T. Devos MD, PhD
Aplastic anaemia is a rare condition characterised by pancytopenia and bone marrow hypocellularity and caused by the immune-mediated destruction of the haematopoietic precursors. The early complications are related to cytopaenias with infections being the major cause of morbi-mortality. The main long-term issue is clonal evolution to myelodysplastic syndrome or acute leukaemia. The diagnosis relies on exclusion of other causes of pancytopenia and characteristic pathologic findings. Severity is stratified according to peripheral blood counts. Nowadays, the survival of treated patients reaches 80–90%. The treatment of the severe form of aplastic anaemia consists on haematopoietic stem cell transplantation in eligible patients and immunosuppressive therapy in non-transplant candidates. Supportive therapy is an option in frail and/or elderly patients. Here, we define and briefly review the pathogenesis of aplastic anaemia. We propose a diagnostic and therapeutic strategy based on existing literature and experts’ recommendations. We finally report three cases illustrating particular clinical associations with pregnancy, hepatitis and paroxysmal nocturnal haemoglobinuria.
(BELG J HEMATOL 2018;9(3):76–85)
Read moreBJH - 2018, issue Abstract Book BHS, february 2018
T. Demuynck , P. Vandenberghe MD, PhD, G. Verhoef MD, PhD, T. Devos MD, PhD
BJH - 2018, issue Abstract Book BHS, february 2018
L. De Roeck , L. Michaux MD, PhD, K. Debackere , E. Lierman PhD, P. Vandenberghe MD, PhD, T. Devos MD, PhD
BJH - 2018, issue Abstract Book BHS, february 2018
J. Van Ham , M. Delforge MD, PhD, A. Janssens MD, PhD, J. Raddoux , M. Beckers MD, PhD, T. Devos MD, PhD, D. Dierickx MD, PhD, V. Vergote MD, J. Maertens MD, PhD, H. Schoemans MD, PhD, P. Vandenberghe MD, PhD
BJH - volume 8, issue 1, february 2017
T. Devos MD, PhD
The presentations on myeloproliferative neoplasms at this year’s ASH congress were inspiring and innovative. As expected, the main topic in chronic myeloid leukemia (CML) was treatment-free remission (TFR). About 30 oral or poster abstracts on this topic were presented during ASH 2016. An update of the important EURO-SKI trial and new results were presented: TFR-studies after treatment with 1st and 2nd generation tyrosine kinase inhibitors (TKI), as well as TFR-studies after both first and second attempts of TKI-discontinuation. While stopping TKI-treatment is considered in CML, starting new treatments and when to start was the main topic regarding the BCR-ABL negative MPNs polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF). A wide panoply of MPN-related topics has been presented at San Diego: late-breaking results of the second-generation JAK-inhibitor pacritinib in MF, interferon-trials in PV and ET, long-term treatment data of the JAK-TKI momelotinib and ruxolitinib, new molecules and novel molecular data, and finally, a special focus on quality of life (QOL). QOL has been a major topic in CML/MPN since several years. In this paper, some key abstracts on CML and MPN, presented at ASH 2016, are selected and commented. I’m aware that this selection excludes many other abstracts, not reducing their intrinsic value. The aim is to present a broad and representative spectrum of studies on each topic. At the end of the article, some ‘take-home messages’ will be given.
(BELG J HEMATOL 2017;8(1):16–22)
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