Challenges in the diagnosis of thrombotic thrombocytopenic purpura

Siniard, Rance C.; Gangaraju, Radhika; May, Jori E.; Marques, Marisa B.

doi:10.1080/17474086.2023.2265058

Cited by 1 publication

(1 citation statement)

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“…Without question, the thrombocytopenia seen in iTTP is significant and indicative of a profound pathologic mechanism which drives platelet consumption. The removal of ADAMTS13 by antibodies to the enzyme appears to represent the best-known pathologic mediator leading to the observed thrombocytopenia that characterizes iTTP [ 42 ]. These antibodies, as mentioned earlier, bind ADAMTS13 and prevent its enzymatic function against vWF multimers.…”

Section: Importance Of Low A-ipc and Possible Additional Pathologic I...mentioning

confidence: 99%

Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura

Dolin,

Maitta

2024

Biomedicines

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Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in diagnosis and treatment, highlighting the need for rapid and effective diagnostic methods. Additionally, the heterogeneous presentation and varied inciting events of TTP suggest more variation in its mechanism than previously thought, implying three potential pathways rather than the accepted two. The recent discovery of ADAMTS13 conformation as a potential contributor to TTP in addition to the proposal of using the absolute immature platelet count (A-IPC) as a biomarker, present novel areas for monitoring and treatment. A-IPC in particular may serve as a more rapid and accurate diagnostic test to distinguish TTP from non-TTP TMAs and to monitor treatment response and relapse. These considerations highlight the need to further study TTP in order to improve best practices and patient care.

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Section: Importance Of Low A-ipc and Possible Additional Pathologic I...mentioning

confidence: 99%