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Is There a Difference in Occurrence of Complications Between Adults With Hemoglobin SS and Hemoglobin SC Disease: An

Martijn van der Meer1, Kim Velsink1, Wing H Tong2,3

  • 1Erasmus Medical Center, Rotterdam, the Netherlands.

European Journal of Haematology
|June 15, 2026
PubMed

Insights

Sickle cell disease (SCD) complications vary by genotype. Hemoglobin SS (HbSS) patients face more acute issues like vaso-occlusive crises, while Hemoglobin SC (HbSC) patients experience more chronic ocular and pulmonary problems.

Area of Science:

  • Hematology
  • Genetics
  • Internal Medicine

Background:

  • Sickle cell disease (SCD) presents diverse acute and chronic complications.
  • Clinical manifestations of SCD complications differ significantly between major genotypes, Hemoglobin SS (HbSS) and Hemoglobin SC (HbSC).

Purpose of the Study:

  • To provide a comprehensive overview of sickle cell disease complications in adults with HbSS and HbSC genotypes.
  • To compare the types and prevalence of complications between HbSS and HbSC genotypes.
  • To identify research gaps and future research directions.

Main Methods:

  • Systematic review adhering to the PRISMA statement guidelines.
  • Extensive literature search conducted on January 2, 2024.
  • Analysis of 31 studies comparing complications in adult HbSS and HbSC patients.

Main Results:

  • Vaso-occlusive crises (VOCs) are the most frequent acute complication in both HbSS and HbSC genotypes.
  • HbSS patients report more frequent VOCs, indicating a more severe acute clinical course.
  • HbSC patients exhibit a higher prevalence of chronic complications, particularly affecting ocular and pulmonary systems.

Conclusions:

  • Both HbSS and HbSC genotypes are susceptible to progressive organ damage from recurrent ischemia and inflammation.
  • Acute complications are more common in HbSS, whereas chronic complications are more prevalent in HbSC.
  • Understanding genotype-specific complications is crucial for tailored SCD management and research.

Keywords:
adultscomplicationshemoglobin SC diseasesickle cell anemiasickle cell disease

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