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Thrombotic Thrombocytopenic Purpura

 

 

Related narratives: Spleen Anatomy
Laparoscopic Splenectomy for Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura is a microangiopathy in which disseminated microvascular occlusion is associated with the pentad of hemolytic anemia, thrombocytopenia, fever, nephropathy and neurological symptoms. It is more common in women and has a peak incidence in the fourth and fifth decades. The cause of TTP is unclear, and whether the endothelial damage is the primary initiating factor or is secondary to platelet agglutinating agents in plasma is uncertain. The end result of the microvascular occlusion is organ damage. The most common presenting symptoms are neurological and hemorrhagic. The disease most often arises in otherwise healthy people.

Plasmapheresis introduced in 1970 has dramatically reversed the lethality of the condition from 80% mortality to over 80% survival of a first episode of the disease. Up to a third of patients relapse after an initial episode. Platelet transfusion has resulted in deterioration of the condition and is not used for TTP. A variety of drugs has been used to supplement plasmapheresis including steroids, antiplatelet agents, immunoglobulin, vincristine and prostacycline. Some patients with relapsing TTP will respond to splenectomy, but the response is not as reliable as it is for splenectomy in the treatment of idiopathic thrombocytopenic purpura (ITP).

Reference:

Lee: Wintrobe's Clinical Hematology, 10th ed., Copyright 1999 Lippincott Williams & Wilkins, Inc. 1613-1617.


This page was last modified on 12-Dec-2000.