BJH - volume 16, issue 2, april 2025
L. Dassy MD, D. Aktan MD, A. Jaspers MD, PhD, S. Shalchian-Tehran MD, V. Delvaux MD, PhD, F. Maraite MD, F. Pitance MD, J-M. Minon MD, PhD, O. Kaye MD, PhD
ADAMTS13, an important enzyme in the regulation of von Willebrand factor, may be deficient, leading to a thrombotic thrombocytopenic purpura, a subtype of thrombotic microangiopathy causing thrombocytopenia, organ failure and haemolytic anaemia. This condition may also be associated with antiphospholipid syndrome and/or systemic lupus erythematosus. We report a case of ischaemic neurological lesions due to an inaugural systemic lupus erythematosus and antiphospholipid syndrome, with the demonstration of a decreased ADAMTS13 activity without biological signs of thrombotic microangiopathy, associated with a possible COVID-19 trigger.
(BELG J HEMATOL 2025;16(2):70–7)
Read moreBJH - volume 6, issue 4, october 2015
O. Ketelslegers MD, F. Eyskens MD, PhD, F. Boemer PhD, V. Bours MD, PhD, J-M. Minon MD, PhD, B. Gulbis MD, PhD
Although neonatal screening for sickle cell disease is one of the best tools for reducing mortality during infancy and early childhood, it is not part of the approved neonatal screening programme in Belgium. As epidemiological data on sickle cell disease are still incomplete in Belgium, we planned to screen the samples of newborns available in the biggest reference centre for approved neonatal screening in Flanders. From July to December 2013, a total of 18,989 newborns from 36 Flemish maternity wards were systematically screened, representing about 60% of the total number of births in Flanders. For the same period, results of the neonatal screening that is routinely performed for sickle cell disease in three other Belgian centres were collected. Overall, 39,599 newborns were screened, representing about two-thirds of Belgian births for this period. With an incidence of sickle cell disease and sickle cell trait of 1/2,329 and 1/77, respectively, sickle cell disease is the most frequently inherited disease observed in the population tested; the highest incidences were registered in urban areas. In addition, screening techniques identified 122 other clinically significant haemoglobin (Hb) variant carriers (83 for HbC, twenty for HbE, thirteen for HbD-Punjab, and six for HbO-Arab) and two HbC diseases. Carriers of clinically significant Hb variants were observed in almost all the maternity wards included in the study, showing a wide dispersal of populations at risk. These epidemiological data remind us of the warnings and recommendations from the World Health Organization, urging policy-makers to consider the most appropriate strategy to prevent and treat patients with sickle cell disease in Belgium.
(BELG J HEMATOL 2015;6(4): 135–41)
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