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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The following authors declare competing interests with respect to this article. Masanori Matsumoto: Received consultant/advisor fees from Baxalta, Alexion Pharma, Ablynx nv and Kainos Laboratories; lecture fees from Asahi Kasei Pharma, Chugai Pharmaceutical, Alexion Pharma, and Bayer Yakuhin; and research funds from Chugai Pharmaceutical, Bayer Yakuhin, and Baxalta. He has shared patent rights with Alfresa Pharma. Yoshihiro Fujimura: Is an employee of the Japanese Red Cross Kinki Block Blood Center. He also has shared patent rights with Alfresa Pharma. Hideo Wada: Received lecture fees from Alexion Pharma and research fund from Daiichi Sankyo. Koichi Kokame: Received patent royalties through National Cerebral and Cardiovascular Center from the Peptide Institute, Peptides International, Kainos Laboratories, SRL, Alfresa Pharma, and AnaSpec. Yoshitaka Miyakawa: Received consultant/advisor fees from Novartis Pharma, Kyowa Hakko Kirin, Kainos Laboratories, and Fujifilm; lecture fees from Novartis Pharma, Kyowa Hakko Kirin, Alexion Pharma, Chugai Pharmaceutical, Bayer Yakuhin, Daiichi Sankyo, Asahi Kasei Pharma, Kissei Pharmaceutical, Biogen, and Baxalta; manuscript fees from Terumo, Zenyaku Kogyo, and the Japan Blood Products Organization; and research funds from Alexion Pharma and Teijin Pharma. Yasunori Ueda: Received consultant/advisor fees from Ablynx nv and Kainos Laboratories; research found for clinical trial from Eli Lilly Japan, Sumitomo Dainippon Pharma, Celgene, Symbio Pharmaceutical, Astellas Pharma, Inc. and Eisai. Takanori Moriki: Is an employee at the Bank of Tokyo-Mitsubishi UFJ, Ltd. Medical Center, Head Office. Toshiyuki Miyata: Received consultant/advisor fees from Alexion Pharma and lecture fees from CSL Behring, LSI Medience, Alexion Pharma, Daiichi Sankyo, and Bayer Yakuhin. He also received patent royalties through National Cerebral and Cardiovascular Center from the Peptide Institute, Peptides International, Kainos Laboratories, SRL, Alfresa Pharma, and AnaSpec. Mitsuru Murata: Received consultant/advisor fees from Pfizer and Otsuka Pharmaceutical; lecture and related fees from Sekisui Medical, LSI Medience, Siemens Healthcare Diagnostics, Sumitomo Dainippon Pharma, Taisho Toyama Pharmaceutical, Tosoh Bioscience, Beckman Coulter, and Alexion Pharma; and research funds from Hitachi High-Technologies, Sanofi, Abbott Japan, Tosoh, and Shino-Test.
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The Japanese version of these guidelines was published in Jpn J Clin Hematol 2017;58:271–81.
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Matsumoto, M., Fujimura, Y., Wada, H. et al. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol 106, 3–15 (2017). https://doi.org/10.1007/s12185-017-2264-7
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DOI: https://doi.org/10.1007/s12185-017-2264-7