KEGG   DISEASE: Thrombotic thrombocytopenic purpura
Entry
H00225                      Disease                                

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
Immune system disease; Cardiovascular disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Hematologic diseases
   H00225  Thrombotic thrombocytopenic purpura
Human diseases in ICD-11 classification [BR:br08403]
 03 Diseases of the blood or blood-forming organs
  Coagulation defects, purpura or other haemorrhagic or related conditions
   3B64  Thrombocytopenia
    H00225  Thrombotic thrombocytopenic purpura
Gene
ADAMTS13 [HSA:11093] [KO:K08627]
Drug
Ticlopidine hydrochloride [DR:D01028]
Caplacizumab [DR:D11160]
Other DBs
ICD-11: 3B64.14
ICD-10: D69.2
OMIM: 274150
Reference
  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
Reference
  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
Reference
  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
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