USMLE (Fach) / Kaplan - Pathology (Lektion)
Rare disorder of hemostasis in which there is a widespread intravascular formation of fibrin-platelet thrombi. It is sometimes associated with an acquired or inherited deficiency of the enzyme ADAMTS13, responsible for cleaving large multimers of von Willebrand factor.
- Often affects adult women
- Inclusion criteria: Microangiopathic hemolytic anemia and thrombocytopenia, with or without renal failure or neurological abnormalities.
- Widespread formation of platelet thrombi with fibrin (hyaline thrombi) leading to intravascular hemolysis (thrombotic microangiopathy)
- Decreased platelet count, prolonged bleeding time
- Normal prothrombin time and partial thromboplastin time
- Peripheral blood smear: thrombocytopenia, schistocytes, reticulocytosis
Treatment: plasma exchange
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