Background: This monocentric study conducted at the University Hospital of Essen aims to describe maternal and fetal/neonatal outcomes in sickle cell disease (SCD) documented between 1996 to 2021 (N = 53), reflecting the largest monocentric analysis carried out in Germany. Methods/Results: 46 pregnancies in 22 patients were followed. None of the patients died. In total, 35% (11/31) of pregnancies were preterm. 15 pregnancies in eight patients were conceived on hydroxycarbamide (HC), of which nine had a successful outcome and three were terminated prematurely. There was no difference regarding the rate of spontaneous abortions in patients receiving HC compared to HC-naive patients prior to conception. In patients other than HbS/C disease, pregnancies were complicated by vaso-occlusive crises (VOCs)/acute pain crises (APCs) (96%, 23/24); acute chest syndrome (ACS) (13%, 3/24), transfusion demand (79%, 19/24), urinary tract infections (UTIs) (42%, 10/24) and thromboembolic events (8%, 2/24). In HbS/C patients complications included: VOCs/APCs (43%, 3/7; ACS: 14%, 1/7), transfusion demand (14%, 1/7), and UTIs (14%, 1/7). Independent of preterm deliveries, a significant difference with respect to neonatal growth in favor of neonates from HbS/C mothers was observed. Conclusion: Our data support the results of previous studies, highlighting the high rate of maternal and fetal/neonatal complications in pregnant SCD patients.
Der Begriff Sichelzellkrankheit umfasst eine Gruppe von Erkrankungen, die durch Sichelzellhämoglobin (HbS) allein oder in Kombination mit anderen Varianten des β-Globin-Locus verursacht werden. Hierbei besteht das Hämoglobin (Hb) zu über 50 % aus HbS. In Deutschland gibt es durch die verstärkte Migration eine zunehmende Anzahl von Patienten mit Sichelzellkrankheit, doch die Kenntnis der Erkrankung und der verschiedenen Genotypen ist noch zu gering. Ziel dieses Beitrags ist es, eine Übersicht über die wichtigsten Krankheitsbilder und deren Besonderheiten zu geben und auf neue Therapieansätze einzugehen.
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