BJH - volume 15, issue 1, february 2024
G. Vertenoeil MD, PhD
SUMMARY
Once again, this year, the ASH Congress provided a wealth of information on myeloproliferative neoplasms (MPNs). In the field of classical Phi-negative MPNs, studies that could potentially influence our practice in the short and medium terms, with a focus on molecular responses and disease modification were selected. In the field of chronic myeloid leukaemia, studies providing additional information on Asciminib as well as strategies to improve the TFR rate in patients were selected. Finally, a study with promising results in the field of MDS/MDN overlap syndromes and chronic neutrophilic leukaemia was included.
(BELG J HEMATOL 2024;15(1):9–12)
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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
SUMMARY
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)
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