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• W4387114568 abstract "ABSTRACTIntroduction Immune-mediated TTP (iTTP) is a rare condition without pathognomic signs and symptoms. For this reason, the diagnosis of iTTP may be delayed or even missed, with potentially catastrophic consequences.Areas covered The authors performed an extensive literature review on the diagnosis of iTTP and its challenges combined with their own experience in a referral center for patients with iTTP.Expert opinion Although a definitive diagnosis of iTTP depends on the ADAMTS13 activity result, timely testing is rarely available at many centers to which patients present. If less complex tests were to become available, they would decrease the chances of late and/or missed diagnoses of iTTP throughout the world. While clinical scores to estimate the likelihood of iTTP exist, they are not well known, and can be misleading if used in the wrong context. Furthermore, the three scoring systems (PLASMIC, Bentley and French) only moderately correlate with each other, which furthers complicates the landscape. The existence of these scores and how they should be used in practice is but one opportunity that can be seized through more robust programs to educate nonspecialist clinicians on how to recognize and treat patients with iTTP.KEYWORDS: Thrombotic thrombocytopenic purpuraThrombotic microangiopathyTTPADAMTS13TTP diagnosisPLASMIC scoreTTP scoring systemsMAHADisclaimerAs a service to authors and researchers we are providing this version of an accepted manuscript (AM). Copyediting, typesetting, and review of the resulting proofs will be undertaken on this manuscript before final publication of the Version of Record (VoR). During production and pre-press, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal relate to these versions also. Declaration of InterestThe authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.Reviewer DisclosuresPeer reviewers on this manuscript have no relevant financial or other relationships to disclose.Article HighlightsTTP is a life-threatening condition who presents with nonspecific signs and symptoms.Acquired TTP is auto-immune and often called iTTP.The diagnosis of iTTP is hampered by its rarity and lack of pathognomonic features.There are scoring systems to determine the likelihood of iTTP but there is no good correlation between them.Confirmation of the diagnosis of iTTP depends on the ADAMTS13 activity assay.Additional informationFundingThis paper was not funded." @default.
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• W4387114568 date "2023-09-28" @default.
• W4387114568 modified "2023-10-16" @default.
• W4387114568 title "Challenges in the diagnosis of thrombotic thrombocytopenic purpura" @default.
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• W4387114568 doi "https://doi.org/10.1080/17474086.2023.2265058" @default.
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