H00225 | |
H number | H00225 |
Name | Thrombotic thrombocytopenic purpura; Moschcowitz disease; Schulman-Upshaw syndrome |
Description | Thrombotic thrombocytopenic purpura (TTP) is caused by mutation in the ADAMTS13 gene and characterized by microangiopathic hemolytic anemia and thrombocytopenia. The idiopathic forms were found to have acquired autoantibody inhibitors of the ADAMTS13 metalloprotease. |
Category | Cardiovascular disease |
Network | - |
Gene | ADAMTS13 [HSA:11093] [KO:K08627] |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Ticlopidine hydrochloride [DR:D01028] Caplacizumab [DR:D11160] |
Comment | - |
Other DBs | ICD-11: 3B64.14 ICD-10: D69.2 OMIM: 274150 |
Reference | PMID:18574040 AUTHORS Sadler JE TITLE Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. JOURNAL Blood 112:11-8 (2008) DOI:10.1182/blood-2008-02-078170 PMID:17666281 AUTHORS Tsai HM TITLE Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency. JOURNAL Hematol Oncol Clin North Am 21:609-32, v (2007) DOI:10.1016/j.hoc.2007.06.003 PMID:17124092 AUTHORS Sadler JE TITLE Thrombotic thrombocytopenic purpura: a moving target. JOURNAL Hematology Am Soc Hematol Educ Program 415-20 (2006) DOI:10.1182/asheducation-2006.1.415 |