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International Journal of Hematology
Published in: International Journal of Hematology 1/2017

01-07-2017 | Guide Line

Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan

Authors: Masanori Matsumoto, Yoshihiro Fujimura, Hideo Wada, Koichi Kokame, Yoshitaka Miyakawa, Yasunori Ueda, Satoshi Higasa, Takanori Moriki, Hideo Yagi, Toshiyuki Miyata, Mitsuru Murata, For TTP group of Blood Coagulation Abnormalities Research Team, Research on Rare and Intractable Disease supported by Health, Labour, and Welfare Sciences Research Grants

Published in: International Journal of Hematology | Issue 1/2017

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Abstract

Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP. In the present guidelines, we have taken all of these criteria into consideration. TTP is classified as acquired if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are detected. Fresh-frozen plasma (FFP) transfusion is performed in patients with congenital TTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with acquired TTP to supplement ADAMTS13 and remove anti-ADAMTS13 autoantibodies. To suppress autoantibody production, corticosteroid therapy may be administered in conjunction with plasma exchange. Recent reports show that the monoclonal anti-CD-20 antibody rituximab is effective in patients with refractory or relapsed TTP.
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Metadata
Title
Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan
Authors
Masanori Matsumoto
Yoshihiro Fujimura
Hideo Wada
Koichi Kokame
Yoshitaka Miyakawa
Yasunori Ueda
Satoshi Higasa
Takanori Moriki
Hideo Yagi
Toshiyuki Miyata
Mitsuru Murata
For TTP group of Blood Coagulation Abnormalities Research Team, Research on Rare and Intractable Disease supported by Health, Labour, and Welfare Sciences Research Grants
Publication date
01-07-2017
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 1/2017
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-017-2264-7

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