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Rheology of red blood cells in patients with HbC disease

Nathalie Lemonne1, Marie Billaud1, Xavier Waltz2,3,4

  • 1Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, 97159 Pointe-à-Pitre, Guadeloupe.

Clinical Hemorheology and Microcirculation
|October 23, 2014
PubMed

Insights

Hemoglobin C disease (CC) patients show fewer severe complications than sickle cell anemia (SS) patients, despite abnormal red blood cell (RBC) properties. Higher RBC oxygen transport effectiveness in CC may explain their lower complication risk.

Area of Science:

  • Hematology
  • Vascular Biology
  • Genetics

Background:

  • Hemoglobin C disease (CC) is generally less severe than sickle cell anemia (SS) or sickle cell hemoglobin C disease (SC).
  • Understanding the hemorheological and clinical differences is crucial for patient management.

Purpose of the Study:

  • To compare hematological, biochemical, hemorheological, and clinical characteristics of CC patients with SS, SC, and healthy (AA) individuals.
  • To investigate the relationship between red blood cell (RBC) properties and clinical outcomes in CC.

Main Methods:

  • Measured blood viscosity, hematocrit-to-blood viscosity ratio (HVR), RBC deformability, and RBC aggregation.
  • Compared CC patients to SS, SC, and AA individuals.

Main Results:

  • CC and SC exhibited higher blood viscosity and lower HVR than AA. CC had higher HVR than SS.
  • CC patients presented with rigid, hyperchromic RBCs and abnormal RBC aggregation (low index, high strength).
  • Despite hemorheological issues, CC patients had no vaso-occlusive crises or acute chest syndrome, but all had splenomegaly; some developed retinopathy or otologic disorders.

Conclusions:

  • The higher oxygen transport effectiveness (HVR) in CC compared to SS likely contributes to the reduced risk of severe medical complications.
  • The exact role of hyperviscosity and decreased RBC deformability in CC complications requires further investigation.

Keywords:
HbC diseaseblood viscosityred blood cell deformabilitysickle cell disease

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