Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome Are Distinct Pathologic Entities

Archives of Pathology and Laboratory Medicine - Tập 127 Số 7 - Trang 834-839 - 2003
Gregory A. Hosler1, Ana María Cusumano1, Grover M. Hutchins1
1From the Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Md

Tóm tắt

Abstract Context.—Thrombotic thrombocytopenic purpura (TTP) and the hemolytic uremic syndrome (HUS) share many clinical features and have been difficult to separate into distinct entities. Histologic examination of organs from autopsied patients suggested that TTP and HUS have dissimilar lesions of different severity and distribution. Objective.—To perform a retrospective observational review of autopsied patients with TTP or HUS to compare the nature and severity of the lesions found. Design.—To examine the pathologic features of these conditions, we reviewed all cases among 51 350 indexed autopsies at The Johns Hopkins Hospital (Baltimore, Md) diagnosed with TTP or HUS, and included those showing multiple arteriolar thrombi or their sequela. Results.—The 56 cases that met the inclusion criteria fell into 2 distinct groups, based on distribution and severity of arteriolar lesions. In 25 patients classified as having TTP, platelet-rich thrombi were present—in decreasing severity—in heart, pancreas, kidney, adrenal gland, and brain. In 31 patients with HUS, fibrin/red cell–rich thrombi were present, largely confined to the kidney and often severe, and only 6 cases showed pancreas involvement, 4 adrenal gland involvement, 2 brain involvement, and 1 heart involvement. Conclusion.—Despite similar clinical features and therapeutic approaches, TTP and HUS each have a characteristic constellation of histopathologic findings. This observation suggests that TTP and HUS are 2 distinct disease entities with different pathophysiologies, and that they do not represent a spectrum of the same disease process.

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Tài liệu tham khảo

Moschcowitz, 1924, Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease., Proc N Y Pathol Soc, 24, 21

Singer, 1947, Thrombotic thrombocytopenia purpura., Blood, 2, 542, 10.1182/blood.V2.6.542.542

Gasser, 1955, Hamolytisch-uramische Syndrome: Bilaterale Nierenrindennekrosen beiakuten erworbenen hamolytischen Anamien., Schweiz Med Wochenschr, 85, 905

Remuzzi, 1987, HUS and TTP: variable expression of a single entity., Kidney Int, 32, 292, 10.1038/ki.1987.206

Kaplan, 1978, The hemolytic-uremic syndrome is a syndrome [editorial]., N Engl J Med, 298, 964, 10.1056/NEJM197804272981710

Wardle, 1983, “Thrombotic micro-angiopathy” [letter]., Clin Nephrol, 20, 323

Furlan, 1998, von Willebrand factor–cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome., N Engl J Med, 339, 1578, 10.1056/NEJM199811263392202

Tsai, 1998, Antibodies to von Willebrand factor–cleaving protease in acute thrombotic thrombocytopenic purpura., N Engl J Med, 339, 1585, 10.1056/NEJM199811263392203

Gianantonio, 1973, The hemolytic-uremic syndrome., Nephron, 11, 174, 10.1159/000180229

Sheth, 1986, Neurological involvement in hemolytic-uremic syndrome., Ann Neurol, 19, 90, 10.1002/ana.410190120

Baehr, 1936, An acute febrile anemia and thrombocytopenic purpura with diffuse platelet thromboses of capillaries and arterioles., Trans Assoc Am Physicians, 65, 43

Asada, 1985, Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen., Thromb Res, 38, 469, 10.1016/0049-3848(85)90180-X

Ridolfi, 1979, The heart and cardiac conduction system in thrombotic thrombocytopenic purpura: a clinicopathologic study of 17 autopsied patients., Ann Intern Med, 91, 357, 10.7326/0003-4819-91-3-357

Morel-Maroger, 1979, Prognostic importance of vascular lesions in acute renal failure with microangiopathic hemolytic anemia (hemolytic-uremic syndrome): clinicopathologic study in 20 adults., Kidney Int, 15, 548, 10.1038/ki.1979.70

Moake, 1998, Moschcowitz, multimers and metalloprotease [editorial]., N Engl J Med, 339, 1629, 10.1056/NEJM199811263392210

Bell, 1991, Improved survival in thrombotic thrombocytopenic purpura–hemolytic uremic syndrome: clinical experience in 108 patients., N Engl J Med, 325, 398, 10.1056/NEJM199108083250605

Baker, 2000, Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome., Curr Opin Pediatr, 12, 23, 10.1097/00008480-200002000-00005

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Archives of Pathology and Laboratory Medicine

Tập 127 Số 7

834-839

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