Thrombotic thrombocytopenic purpura historical perspective
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Saeedeh Kowsarnia M.D.[2]
Overview
Historical Perspective
- In 1924, Eli Moschcowitz first describe TTP with a pentad of presentation (petechiae, anemia, and pallor followed by paralysis and coma) .[1]
- In 1960, plasma infusion was introduced as a part of the treatment of a 8-year old patient with chronic anemia and thrombocytopenia by Schulman and colleagues [2].
- In 1978, Upshaw first described the hereditary aspect of the disease which lacked an enzyme in the blood and responded to plasma transfusion [3].
- In 1979, Rennard & Abe proposed the use of the name Upshaw-Schulman syndrome to define the disease with these clinical features who responded to plasma infusion [4].
- In 1966, the new criteria for the diagnosis of TTP as the “pentad” of clinical and laboratory features was suggested. The criteria included thrombocytopenia, microangiopathic hemolytic anemia (fragmentation of RBCs or schistocytes) in the peripheral blood smear, neurological signs and symptoms, renal failure, and fever .
- Moschcowitz ascribed the disease (incorrectly) to a toxic cause. Moschcowitz noted that his 16 year-old patient had anemia; petechiae; microscopic hematuria; and at autopsy, disseminated microvascular thrombi. Since that time, the pathophysiology, etiology, and medical management of TTP has expanded.
References
- ↑ Moschcowitz E (1924). "An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease". Proc NY Pathol Soc. 24: 21–4. Reprinted in Mt Sinai J Med 2003;70(5):322-5, PMID 14631522.
- ↑ SCHULMAN I, PIERCE M, LUKENS A, CURRIMBHOY Z (1960). "Studies on thrombopoiesis. I. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency". Blood. 16: 943–57. PMID 14443744.
- ↑ Upshaw, Jefferson D. (1978). "Congenital Deficiency of a Factor in Normal Plasma That Reverses Microangiopathic Hemolysis and Thrombocytopenia". New England Journal of Medicine. 298 (24): 1350–1352. doi:10.1056/NEJM197806152982407. ISSN 0028-4793.
- ↑ Rennard S, Abe S (1979). "Decreased cold-insoluble globulin in congenital thrombocytopenia (Upshaw-Schulman syndrome)". N Engl J Med. 300 (7): 368. doi:10.1056/NEJM197902153000718. PMID 759902.