The German sickle cell disease registry reveals a surprising risk of acute splenic sequestration and an increased transfusion requirement in patients with compound heterozygous sickle cell disease HbS/β-thalassaemia and no or low HbA expression.

doi:10.1111/ejh.14259

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The German sickle cell disease registry reveals a surprising risk of acute splenic sequestration and an increased transfusion requirement in patients with compound heterozygous sickle cell disease HbS/β-thalassaemia and no or low HbA expression.

European journal of haematology - 1 Oct 2024

Allard Pierre, Tagliaferri Laura, Weru Vivienn, Cario Holger, Lobitz Stephan, Grosse Regine, Bleeke Matthias, Oevermann Lena, Hakimeh Dani, Jarisch Andrea, Kopp-Schneider Annette, Kulozik Andreas E, Kunz Joachim B, Lassay Lisa

Abstract excerpt

Patients with sickle cell disease (SCD) in Germany exhibit a substantial genetic diversity in the β-globin genotype. Data collected by the national German SCD registry reflect this diversity and allowed us to analyze the phenotypes associated with different SCD genotypes. Our study focused on 90 patients with HbS/β-thalassaemia (HbS/β-thal) and compared these to patients with HbSS and HbSC. Patients with...

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PMID: 38946051
DOI: 10.1111/ejh.14259

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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