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Year : 2022  |  Volume : 13  |  Issue : 3  |  Page : 111-117

Approaches to acquired thrombotic thrombocytopenic purpura management in Saudi Arabia

1 Department of Oncology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
2 Department of Adult Hematology, King Faisal Specialist Hospital and Research Center, Madinah, Saudi Arabia
3 Department of Adult Hematology and Stem Cell Transplantation, King Fahad Specialist Hospital, Dammam, Saudi Arabia
4 Department of Hematology and Bone Marrow Transplantation Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
5 Department of Adult Hematology/BMT, King Fahad Medical City, Riyadh, Saudi Arabia
6 Department of Adult Hematology/BMT, King Abdulaziz Medical City, Jeddah, Saudi Arabia
7 Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
8 Department of Adult Hematology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
9 Department of Medical Affairs, Sanofi, Jeddah, Saudi Arabia
10 Department of Medical Affairs, Sanofi, Dubai, UAE
11 Department of Hematology, Faculty of Medicine, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

Correspondence Address:
Dr. Ayman AlHejazi
Department of Oncology, King Abdulaziz Medical City, Riyadh 14611
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joah.joah_46_21

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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.

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