TY  - JOUR
AU  - Allard, Pierre
AU  - Tagliaferri, Laura
AU  - Weru, Vivienn
AU  - Cario, Holger
AU  - Lobitz, Stephan
AU  - Grosse, Regine
AU  - Bleeke, Matthias
AU  - Oevermann, Lena
AU  - Hakimeh, Dani
AU  - Jarisch, Andrea
AU  - Kopp-Schneider, Annette
AU  - Kulozik, Andreas E
AU  - Kunz, Joachim B
TI  - 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.
JO  - European journal of haematology
VL  - 113
IS  - 4
SN  - 0902-4441
CY  - Oxford
PB  - Wiley-Blackwell
M1  - DKFZ-2024-01402
SP  - 501-509
PY  - 2024
N1  - 2024 Oct;113(4):501-509
AB  - 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 HbS/β-thal were classified into three groups: HbS/β0-thal (no HbA), HbS/β+-thal (HbA < 14
KW  - anemia (Other)
KW  - sickle cell (Other)
KW  - thalassemia (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:38946051
DO  - DOI:10.1111/ejh.14259
UR  - https://inrepo02.dkfz.de/record/291428
ER  -