Coagulation changes in sickle cell disease in early childhood

Area of Science:

• Hematology
• Pediatrics
• Vascular Biology

Background:

• Sickle cell disease (SCD) is a genetic blood disorder.
• Coagulation abnormalities are common in SCD patients.
• The role of coagulation in SCD pain crises is not fully understood.

Purpose of the Study:

• To investigate coagulation system parameters in children with SCD.
• To compare coagulation in steady-state SCD, painful crisis, and healthy controls.
• To determine if coagulation changes contribute to SCD pain crisis onset.

Main Methods:

• Coagulation tests including PT, PTT, TT, and fibrinogen were performed.
• Platelet aggregation studies using ADP, collagen, adrenaline, and ristocetin were conducted.
• Measurements were taken in children with SCD during steady state and painful crisis, compared to controls.

Main Results:

• No significant differences in most coagulation parameters between SCD patients and controls.
• Abnormal platelet aggregation responses to ristocetin were observed in all SCD patients.
• During painful crisis, fibrinogen and ristocetin-cofactor (Ri-Cof) levels increased, with enhanced platelet aggregation to ADP and adrenaline by day 3.

Conclusions:

• Abnormal ristocetin-induced platelet aggregation is a feature of SCD.
• Coagulation changes during SCD painful crisis appear secondary to crisis etiology.
• Stasis and acute-phase proteins are likely triggers for these secondary coagulation alterations.