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

XML Print

Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

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:   (515 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]   (235 Downloads)    
Type of Study: case report | Subject: Special
Received: 2019/03/15 | Accepted: 2020/12/22 | Published: 2021/06/20

Add your comments about this article : Your username or Email:

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

© 2022 CC BY-NC 4.0 | Iranian Journal of Pediatric Hematology and Oncology

Designed & Developed by : Yektaweb