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Elevated hypercoagulability markers in hemoglobin SC disease.

Marina P Colella1, Erich V de Paula1, João A Machado-Neto1

  • 1Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.

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Summary
This summary is machine-generated.

Hemoglobin SC disease patients show a hypercoagulable state with elevated coagulation markers compared to healthy individuals. This blood clotting activation is less intense than in sickle cell anemia but linked to complications like retinopathy.

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Area of Science:

  • Hematology
  • Vascular Biology
  • Thrombosis

Background:

  • Hemoglobin SC disease is a common hemoglobinopathy with poorly understood hemostatic alterations.
  • Increased risk of thromboembolic events is suspected, but specific coagulation studies are lacking.

Purpose of the Study:

  • To evaluate coagulation activation markers in adult patients with Hemoglobin SC disease.
  • To compare these markers with those in sickle cell anemia patients and healthy controls.

Main Methods:

  • Cross-sectional observational study design.
  • Analysis of coagulation activation markers, endothelial activation, and inflammation markers in 56 Hemoglobin SC patients, 39 sickle cell anemia patients, and 27 healthy controls.
  • Patients were in steady state and not taking hydroxyurea.

Main Results:

  • Hemoglobin SC patients exhibited significantly upregulated tissue factor, thrombin-antithrombin complex, and D-dimer compared to controls.
  • These markers were elevated but lower in Hemoglobin SC disease than in sickle cell anemia.
  • Endothelial activation and inflammation markers were significantly elevated in Hemoglobin SC patients, similar to sickle cell anemia patients.
  • Higher hemolytic activity and inflammation correlated with increased coagulation activation.
  • Hemostatic activation was associated with retinopathy and osteonecrosis in Hemoglobin SC disease.

Conclusions:

  • Hemoglobin SC disease patients present a hypercoagulable state.
  • This hypercoagulability is linked to endothelial activation, inflammation, and chronic complications.
  • The hemostatic activation in Hemoglobin SC disease is less pronounced than in sickle cell anemia.