Thrombotic Thrombocytopenic Purpura

Classically a pentad:

Thrombocytopenia
Fragmentation Hemolysis
Fever
Neurological Dysfunction
Renal Impairment

Similar in many repects to hemolytic uremic syndrome (sharing thrombocytopenia, microangiopathic hemolytic anemia, renal impairment), but 90 percent mortality.

Pathogensis

Unknown etiology
Associations

Exposure to certain drugs (chemotherapeutics, OCPs)
Pregnancy
Collagen vascular diseases
Cyclosporin immunosuppression

Occlusion of capillaries with microthrombi
Increased calpain, a cyteine protease which affects vWF, increasing platelet aggregation.

Plasma exchange (or infusion)
Platelet transfusions may exacerbate the process

Heme-Onc

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Last modification: July 18, 2000