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Year : 2022  |  Volume : 13  |  Issue : 3  |  Page : 145-153

Burden of acquired thrombotic thrombocytopenic purpura: KSA and UAE expert consensus for improved disease management

1 Dubai Health Authority, Hematology Department, Dubai Hospital, Dubai, UAE
2 Hematology Department, King Fahad Specialist Hospital, Dammam, KSA
3 Division of Hematology & HCT, Department of Oncology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
4 Department of Adult Hematology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
5 Hematology Department, Tawam Hospital, Abu Dhabi, UAE
6 Hematology Department, King Khalid University Hospital, Riyadh, KSA
7 Department of Hematology, Dubai Health Authority, Mediclinic City Hospital, Dubai, UAE

Correspondence Address:
Prof. Mahmoud Marashi
Mediclinic City Hospital, DHCC, Dubai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joah.joah_149_21

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BACKGROUND: In Saudi Arabia (KSA) and the United Arab Emirates (UAE), only limited epidemiological data and treatment guidance exist for acquired thrombotic thrombocytopenic purpura (aTTP), a rare, life-threatening blood disorder. AIMS: Expert insights from KSA and UAE were used to obtain local epidemiological data, to characterize current disease management and unmet needs, and to formulate recommendations for the improvement of the diagnosis and treatment of aTTP. Costs and socioeconomic burden were a secondary focus. METHODS: Hematologists from KSA and UAE with clinical experience in the diagnosis and management of aTTP individually answered questions on the burden and management of aTTP via an online survey. Based on these insights, a draft consensus was discussed and refined jointly by the hematologists in a live session for each country. Payers provided information on the economic burden and cost of aTTP management. RESULTS: The experts estimate the incidence of aTTP to 5–6 (KSA) and 1–2 (UAE) per 1,000,000 person-years and anticipate it increasing. Most of the presenting patients are aTTP-naive. Recurrent disease is rare. Diagnosis of aTTP should involve ADAMTS13 activity testing. Plasma exchange and immunosuppression are the current standard of care. Key unmet needs include a lack of awareness of aTTP, access to rapid testing and novel treatments to improve outcomes. CONCLUSION: The expert consensus to address the unmet needs and improve aTTP outcomes include increasing aTTP awareness and access to ADAMTS13 testing; the development of national guidelines; and, additionally, strategies to improve patients' long-term quality of life.

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