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- W4366990954 abstract "Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by a hereditary or immune-mediated deficiency of the enzyme ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). TTPs are caused by the following pathophysiological mechanisms: (1) the presence of inhibitory autoantibodies against ADAMTS13; and (2) hereditary mutations of the ADAMTS13 gene, which is present on chromosome 9. In both syndromes, TTP results from a severe deficiency of ADAMTS13, which is responsible for the impaired proteolytic processing of high-molecular-weight von Willebrand factor (HMW-VWF) multimers, which avidly interact with platelets and subendothelial collagen and promote tissue and multiorgan ischemia. Although the acute presentation of the occurring symptoms in acquired and hereditary TTPs is similar (microangiopathic hemolytic anemia, thrombocytopenia, and variable ischemic end-organ injury), their intensity, incidence, and precipitating factors are different, although, in both forms, a severe ADAMTS13 deficiency characterizes their physiopathology. This review is aimed at exploring the possible factors responsible for the different clinical and pathological features occurring in hereditary and immune-mediated TTPs." @default.
- W4366990954 created "2023-04-27" @default.
- W4366990954 creator A5004284893 @default.
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- W4366990954 creator A5083235783 @default.
- W4366990954 date "2023-04-25" @default.
- W4366990954 modified "2023-09-26" @default.
- W4366990954 title "Immune and Hereditary Thrombotic Thrombocytopenic Purpura: Can ADAMTS13 Deficiency Alone Explain the Different Clinical Phenotypes?" @default.
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- W4366990954 doi "https://doi.org/10.3390/jcm12093111" @default.
- W4366990954 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37176552" @default.
- W4366990954 hasPublicationYear "2023" @default.
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