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Thrombotic Thrombocytopenic Purpura
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     To the Editor: In his review of the diagnosis and treatment of thrombotic thrombocytopenic purpura (May 4 issue),1 Dr. George did not mention acetylsalicylic acid, or aspirin. Platelet activation leading to microvascular thrombi is the main pathologic feature of thrombotic thrombocytopenic purpura. Consequently, it is logical to complement plasma exchange and corticosteroids with inhibitors of platelet function. For this reason, aspirin was part of the standard treatment protocols in the two largest studies of plasma exchange.2,3 There have been reports of sudden deterioration and death among patients with thrombotic thrombocytopenic purpura during recovery thrombocytosis because they were not taking platelet inhibitors.4 Antiplatelet agents are usually not recommended for patients with thrombotic thrombocytopenic purpura when bleeding is observed or when they also have severe thrombocytopenia, but it might be advisable for these patients to start treatment with aspirin once platelet counts begin to rise again in response to plasma exchange. Moreover, maintenance treatment with inhibitors of platelet function may prevent relapses of thrombotic thrombocytopenic purpura.5

    Axel C. Matzdorff, M.D.

    Caritasklinik St. Theresia

    66113 Saarbruecken, Germany

    a.matzdorff@caritasklinik.de

    References

    George JN. Thrombotic thrombocytopenic purpura. N Engl J Med 2006;354:1927-1935.

    Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients. N Engl J Med 1991;325:398-403.

    Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 1991;325:393-397.

    Gordon LI, Kwaan HC, Rossi EC. Deleterious effects of platelet transfusions and recovery thrombocytosis in patients with thrombotic microangiopathy. Semin Hematol 1987;24:194-201.

    Bobbio-Pallavicini E, Gugliotta L, Centurioni R, et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP): results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 1997;82:429-435.

    The author replies: I agree that aspirin warrants consideration for the treatment of thrombotic thrombocytopenic purpura. Although I generally use aspirin only for patients with neurologic or cardiac ischemic complications, it is a logical consideration for a disorder characterized by platelet-dependent arteriolar thrombosis. All patients in the randomized trial by the Canadian Apheresis Group that compared plasma exchange with plasma infusion1 were treated with acetylsalicylic acid (together with dipyridamole), and the use of aspirin remains a standard practice of the Canadian Apheresis Group, if the patient is not bleeding and the platelet count is more than 20,000 per cubic millimeter (LeBlond PF, Rock GA: personal communication). However, a large case series,2 published at the same time as the Canadian clinical trial, reported outcomes among patients who underwent plasma exchange without the use of acetylsalicylic acid that were similar to the outcomes among patients in the Canadian trial. A more recent randomized trial3 reported a nonsignificant reduction in mortality when aspirin and dipyridamole were given in addition to plasma exchange and corticosteroids, as compared with plasma exchange and corticosteroids alone. Low-dose aspirin is recommended by the British Committee for Standards in Haematology for patients with platelet counts greater than 50,000 per cubic millimeter.4

    James N. George, M.D.

    University of Oklahoma Health Sciences Center

    Oklahoma City, OK 73190

    james-george@ouhsc.edu

    References

    Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 1991;325:393-397.

    Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients. N Engl J Med 1991;325:398-403.

    Bobbio-Pallavicini E, Gugliotta L, Centurioni R, et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP): results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 1997;82:429-435.

    Allford SL, Hunt BJ, Rose P, Machin S, Haemostasis and Thrombosis Task Force. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 2003;120:556-573.