ArticleBritish journal of haematology2024
The clinical spectrum of HbSC sickle cell disease-not a benign condition.
Article in British journal of haematology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 2 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
18 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Is There a Difference in Occurrence of Complications Between Adults With Hemoglobin SS and Hemoglobin SC Disease: An Extended Systematic Review.European journal of haematology · 2026Pooled it
- Vitamin B12 Deficiency in Sickle Cell Disease: Method-Driven Estimates and Systematic Diagnostic Misclassification.European journal of haematology · 2026Pooled it
- Baseline characteristics of Ghanaian children and adults enrolled in PIVOT, a randomised clinical trial of hydroxyurea in HbSC disease in sub-Saharan Africa.British journal of haematology · 2024Trial
- Identification of Clinical Sub-Phenotypes of Sickle Cell Disease Using Latent Class Analysis.American journal of hematology · 2026Article
- EHA Guidelines on the management of children and adults with hemoglobin SC disease.HemaSphere · 2026Article
- TTP-Like Syndrome and Subsequent Non-Aneurysmal Subarachnoid Hemorrhage in HbSC Disease: A Case Report.Clinical case reports · 2026Article
- Hydroxyurea (hydroxycarbamide) use in adults with haemoglobin SC disease: A real-world study in Quebec.British journal of haematology · 2026Article
- Hydroxyurea (hydroxycarbamide) in haemoglobin SC disease: Moving from therapeutic hesitation to evidence-guided care.British journal of haematology · 2026Article
- Positive neurological outcome after acute encephalopathy and multifocal stroke after early red blood cell exchange transfusion in a patient with hemoglobin SC sickle cell disease.Proceedings (Baylor University. Medical Center) · 2026Article
- Iron, arginine, and redox metabolism in peripheral blood mononuclear cells distinguishes sickle cell disease and pulmonary hypertension.HemaSphere · 2026Article
- Review
- Genotype Differences and Hydroxyurea Utilization Among Adults With Moderate to Severe Sickle Cell Disease.Pharmacotherapy · 2026Article
- Atypical Overt Hemorrhagic Stroke in an Adult With Sickle Cell-Hemoglobin C Disease: A Report of a Rare Case.Cureus · 2026Article
- A mouse model for hemoglobin SC disease recapitulates characteristic human pathologies.Blood advances · 2025Article
- Article
- Article
- The phenotypes of sickle cell disease: strategies to aid the identification of undiagnosed patients in the Italian landscape.Italian journal of pediatrics · 2025Review
- Health-Related Quality of Life Measurement in Adults With Sickle Cell Disease in Steady State: Experience of One French Reference Center.Mediterranean journal of hematology and infectious diseases · 2025Article
Corrections and comments
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Authors and funding
13 authors.
Funding
Abstract
Sickle cell disease (SCD) includes a group of heterogenous disorders that result in significant morbidities. HbSS is the most common type of SCD and HbSC is the second most common type of SCD. The prevalence of HbSC disease in the United States and United Kingdom is ~1 in 7174 births and 1 in 6174 births respectively. Despite its frequency, however, HbSC disease has been insufficiently studied and was historically categorized as a more 'mild' form of SCD. We conducted this study of HbSC disease as part of the NHLBI funded Sickle Cell Disease Implementation Consortium (SCDIC). The SCDIC registry included 2282 individuals with SCD, ages 15-45 years of whom 502 (22%) had HbSC disease. Compared with people with sickle cell anaemia (SCA), the study found that people with HbSC disease had a higher frequency of splenomegaly (n (%) = 169 (33.7) vs. 392 (22.1)) and retinopathy (n (%) = 116 (23.1) vs. 189 (10.6)). A Many people with HbSC also had avascular necrosis (n (%) = 112 (22.3)), pulmonary embolism (n (%) = 43 (8.6)) and acute chest syndrome (n (%) = 228 (45.4)) demonstrating significant disease severity. HbSC disease is more clinically severe than was previously recognized and deserves additional evaluation and targeted treatments.
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