Hemostatic alterations accompanying sickle cell pain crises

Area of Science:

• Hematology
• Vascular Biology
• Thrombosis

Background:

• Sickle-cell disease (SSD) is a genetic blood disorder characterized by abnormal hemoglobin.
• Vaso-occlusive crises are a hallmark of SSD, leading to significant morbidity.
• The hemostatic system's role during SSD crises requires further elucidation.

Purpose of the Study:

• To investigate the sequential hemostatic alterations during sickle-cell disease crises.
• To assess the impact of crises on platelet count and fibrinogen metabolism.
• To explore the potential utility of antiplatelet agents in managing SSD-related hemostatic abnormalities.

Main Methods:

• Longitudinal weekly monitoring of three patients with sickle-cell disease over 1.5-2 years.
• Assessment of hemostatic parameters, including plasma fibrinogen complexes, platelet counts, and fibrinogen breakdown products.
• Observation of clinical status and correlation with laboratory findings.

Main Results:

• Crises were associated with intravascular fibrin formation and significant platelet count fluctuations.
• Increased high-molecular-weight fibrinogen complexes and decreased platelet counts preceded or coincided with crisis onset.
• Fibrinogen levels and platelet counts subsequently rose, with peak levels occurring post-crisis.
• Fibrinogen breakdown products increased as crises subsided.
• Hemostatic abnormalities correlated with clinical status, normalizing during asymptomatic periods.

Conclusions:

• Sickle-cell disease crises involve dynamic changes in coagulation and platelet dynamics.
• These findings suggest a potential role for agents affecting platelet function, such as aspirin and dipyridamole, in mitigating crisis-associated hemostatic disturbances.
• Further research into antiplatelet therapies for SSD management is warranted.