TTP/HUSに対する血漿交換などの各種治療法

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Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) have considerable overlap in their clinical presentations and markedly variable clinical courses. Indeed, HUS is now regarded as part of a spectrum of thrombotic microangiopathies (TMA), one extreme consisting of TTP with predominant neurologic findings and minimal renal abnormality, and the other profound renal dysfunction with no or minimal CNS pathology. The hallmarks of TMA are microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. The clinical presentation of TTP is characterized by a pentad of finding : MAHA, thrombocytopenia, neruologic abnormalities, renal dysfunction, and fever. The clinical presentation of HUS is characterized by MAHA, renal dysfunction, and thrombocytopenia. TTP/HUS is a hematologic emergency. Patients will often need ICU admission because of profound anemia, shock, lactic acidosis, respiratory failure, or deteriorating neurologic function. In many cases, intubation and mechanical ventilation may be required because of ventilatory failure. Once a diagnosis of TTP/ HUS is made, plasma exchange is the therapy of choice. Plasma exchange should be performed daily until evidence indicates the disease is in remission. The most sensitive indicator of response is the platelet count, and LDH. The usual course of plasma exchange is 5 to 10 days. Plasma infusion therapy is useful for many patients. In general, platelet infusion therapy is contraindicated in TTP/HUS, based on clinical evidence that it leads to relapse. Specific therapy by antiplatelet agents was found to be of no benefit.
日本アフェレシス学会の論文
1999-11-30

TTP/HUSに対する血漿交換などの各種治療法

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