• Rinsho Ketsueki · Jan 2020

    [Diagnosis and treatment of thrombotic thrombocytopenic purpura].

    • Masayuki Kubo and Masanori Matsumoto.
    • Department of Blood Transfusion Medicine, Nara Medical University.
    • Rinsho Ketsueki. 2020 Jan 1; 61 (5): 529-535.

    AbstractThrombotic thrombocytopenic purpura (TTP) is a life-threatening disease in which platelets are consumed and thrombotic microangiopathy develops in multiple organs due to a severe deficiency of the metalloproteinase, ADAMTS13. TTP should be suspected in any case associated with thrombocytopenia and hemolytic anemia; TTP can be diagnosed in cases of profound reduction in ADAMTS13 activity (to <10% of the normal level). Congenital TTP involves mutations in the ADAMTS13 gene, whereas acquired or autoimmune TTP results from the actions of inhibitory autoantibodies against the ADAMTS13 protein. Plasma exchange together with corticosteroids is an effective treatment for acquired TTP; plasma exchange removes autoantibodies and provides ADAMTS13 supplementation, whereas corticosteroids further suppress autoantibody generation. Rituximab was recently approved in Japan for use in refractory or relapsing TTP. Likewise, caplacizumab, an anti-von Willebrand factor, may contribute to disease control and overall survival by preventing ongoing thrombosis and acute end-organ damage.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…