Severe Thrombocytopenia in Sickle Cell Crisis

Abstract

Thrombocytopenia is unusual in sickle cell disease. We present the case of a child with homozygous sickle cell disease who experienced life-threatening thrombocytopenia during a prolonged and disabling vasoocclusive episode. Irreversibly sickled cells were conspicuously absent from the peripheral blood at presentation and during the subsequent protracted illness. These observations illustrate the pathophysiology of the intravascular events during vasoocclusive crisis and provide indirect evidence for the consumption of both platelets and irreversibly sickled cells at sites of infarction.

Key Words:

• thrombocytopenia
• vasoocclusive crisis
• sickle cell disease
• irreversibly sickled cells