The clinical spectrum of HbSC sickle cell disease‐not a benign condition

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Registry analysis reveals substantial clinical morbidity in individuals with HbSC sickle cell disease, indicating the condition is not benign.

HbSC disease causes substantial clinical morbidity rather than mild illness, presenting with elevated frequencies of splenomegaly and retinopathy.
Acute chest syndrome affected 45.4% of patients with HbSC disease, while retinopathy developed in 23.1% of individuals during clinical follow-up.
Analysis of registry records from 2282 individuals highlights severe HbSC disease complications, calling for targeted therapies and clinical evaluation.

Nelson et al. (2024) studied this question.

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Registry analysis reveals substantial clinical morbidity in individuals with HbSC sickle cell disease, indicating the condition is not benign.