Open AccessThe Challenges in Diagnosis and Management of Acquired Thrombotic Thrombocytopenic Purpura: Consensus Report from Three Gulf Countries
Author(s) -
Murtadha Al-Khabori,
Faisal Al Sayegh,
Hasan A Al Yaseen,
Sabir Hussien,
Amar Lal,
Muna Al Rasheed,
Mohammad Al Bader,
Salam Al Kindi,
Mahmoud Marashi
Publication year - 2022
Publication title -
oman medical journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.395
H-Index - 31
eISSN - 2070-5204
pISSN - 1999-768X
DOI - 10.5001/omj.2022.32
Subject(s) - medicine , thrombotic thrombocytopenic purpura , microangiopathic hemolytic anemia , therapeutic plasma exchange , referral , intensive care medicine , purpura (gastropod) , pediatrics , clinical trial , immunology , family medicine , platelet , ecology , biology
Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare hematological emergency that is characterized by microangiopathic hemolytic anemia (MHA), thrombocytopenia, fever, and multiorgan failure due to autoimmune-mediated deficiency in ADAMTS-13 activity. Currently plasma exchange, with or without steroids, is the frontline option of management of aTTP that should be started promptly once the disorder is clinically-suspected. Besides, immunomodulators were studied in patients with aTTP to achieve stable remission and reduce the risk of relapse in patients with suboptimal response to plasma exchange; however, clinical trials showed equivocal results. in addition, published data on early diagnosis, referral, and treatment patterns of aTTP patients in Gulf Council Countries (GCC) are still lacking. Therefore, the present consensus report aimed to present an overview of aTTP situation in GCC by bringing together a panel of experts from three GCC to share their views on current trends and practice regarding aTTP. The experts discussed challenges including the lack of reliable data regarding the incidence of aTTP in GCC and delayed results of ADAMTS-13 activity testing. Limited patients’ access to tertiary centers and low level of awareness about the aTTP clinical spectrum among general practitioners are other challenges. The experts agreed that there is a need for national and regional consensus regarding the diagnosis and treatment of aTTP in Gulf region.