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- Title
Approaches to acquired thrombotic thrombocytopenic purpura management in Saudi Arabia.
- Authors
AlHejazi, Ayman; AlBeihany, Amal; AlHashmi, Hani; Alzahrani, Hazzaa; Motabi, Ibraheem; El-Hemaidi, Ihab; Alsaleh, Khalid; El Tayeb, Khaled; Rabea, Magdy; Khallaf, Mohamed; Qari, Mohammad
- Abstract
Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening microangiopathy usually characterized by microangiopathic hemolytic anemic, thrombocytopenia, and end-organ ischemia associated with disseminated microvascular platelet-rich thrombi and severe deficiency (activity <10%) of A Disintegrin-like And Metalloprotease with ThromboSpondin Type 1 Motif No. 13 (ADAMTS13). It is a medical emergency, and if left untreated, acute mortality is as high as 90%. This review article is a narrative review based on available literature. In addition, the key discussions of the Kingdom of Saudi Arabia experts and members of "Approaches to aTTP Management" Advisory Board meeting held on October 16, 2020, have been incorporated as expert opinions. It was agreed that treatment should be started based on the presumptive diagnosis and continued until remission or an alternate diagnosis is established. Use of caplacizumab in addition to therapeutic plasma exchange and immunosuppression is recommended in confirmed aTTP episodes.
- Subjects
SAUDI Arabia; HEMOLYTIC anemia treatment; ISCHEMIA; PLASMA exchange (Therapeutics); THROMBOTIC thrombocytopenic purpura; IMMUNOSUPPRESSION; GLYCOPROTEINS; RESEARCH funding; NEEDS assessment; PEPTIDES
- Publication
Journal of Applied Hematology, 2022, Vol 13, Issue 3, p111
- ISSN
1658-5127
- Publication type
Article
- DOI
10.4103/joah.joah_46_21