Report of two unrelated cases of Familial Thrombotic Thrombocytopeic Purpura

نویسندگان
Darbandi , Bahram ID 1
Hajireza , Pantea ID 2
Baghersalimi , Adel ID 3

1 Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran

2 Guilan University of Medical Sciences, Rasht, Iran

3 Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran.

doi
10.18502/ijpho.v11i3.6571
چکیده

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 ...

کلیدواژه‌ها
ADAMTS13, Hemolytic anemia, Thrombotic Thrombocytopenic Purpura