Volume 11, Issue 3 (6-2021)                   Iran J Ped Hematol Oncol 2021, 11(3): 211-215 | Back to browse issues page


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Darbandi B, Baghersalimi A, Hajireza P. Report of two unrelated cases of Familial Thrombotic Thrombocytopeic Purpura. Iran J Ped Hematol Oncol. 2021; 11 (3) :211-215
URL: http://ijpho.ssu.ac.ir/article-1-644-en.html
Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran.
Abstract:   (72 Views)
Thrombotic Thrombocytopenic Purpura (TTP) is a rare microangiopathic disorder characterised by the pentad of microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal disease. Decreased production and/or activity of ADAMTS13 is the cause of this disorder. ADAMTS13 is a metalloproteinase which is responsible of the cleavage of high weight multimers of von Wilebrand factor. Its acquired form is usually seen in adults and is due to antibody formation against the enzyme. But even rarer familial and relapsing forms (Upshow-Scholman syndrome) are due to enzyme underproduction and can be seen in pediatric age group. Here, the authors reported two unrelated cases of familial TTP from Guilan province in the north of Iran.
Full-Text [PDF 199 kb]   (39 Downloads)    
Type of Study: case report | Subject: Special
Received: 2019/03/15 | Accepted: 2020/12/22 | Published: 2021/06/20

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