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

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.
PMID
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Authors

Mayor MeshTerms
Keywords

ADAMTS13

TMA

TTP

Journal Title international journal of hematology
Publication Year Start




PMID- 28550351
OWN - NLM
STAT- Publisher
DA  - 20170527
LR  - 20170527
IS  - 1865-3774 (Electronic)
IS  - 0925-5710 (Linking)
DP  - 2017 May 26
TI  - Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP)
      2017 in Japan.
LID - 10.1007/s12185-017-2264-7 [doi]
AB  - 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.
FAU - Matsumoto, Masanori
AU  - Matsumoto M
AD  - Department of Blood Transfusion Medicine, Nara Medical University, 840
      Shijyo-cho, Kashihara, Nara, 634-8522, Japan. [email protected]
FAU - Fujimura, Yoshihiro
AU  - Fujimura Y
AD  - Japanese Red Cross Kinki Block Blood Center, Ibaraki, Japan.
FAU - Wada, Hideo
AU  - Wada H
AD  - Department of Molecular and Laboratory Medicine, Mie University Graduate School
      of Medicine, Tsu, Japan.
FAU - Kokame, Koichi
AU  - Kokame K
AD  - Department of Molecular Pathogenesis, National Cerebral and Cardiovascular
      Center, Suita, Japan.
FAU - Miyakawa, Yoshitaka
AU  - Miyakawa Y
AD  - Department of General Internal Medicine and Center for Thrombosis and Hemostasis,
      Saitama Medical University Hospital, Iruma, Japan.
FAU - Ueda, Yasunori
AU  - Ueda Y
AD  - Department of Hematology/Oncology, Kurashiki Central Hospital, Kurashiki, Japan.
FAU - Higasa, Satoshi
AU  - Higasa S
AD  - Division of Hematology, Department of Internal Medicine, Hyogo College of
      Medicine, Nishinomiya, Japan.
FAU - Moriki, Takanori
AU  - Moriki T
AD  - Department of Laboratory Medicine, Keio University School of Medicine, Tokyo,
      Japan.
FAU - Yagi, Hideo
AU  - Yagi H
AD  - Department of Hematology, Nara Hospital, Kinki University School of Medicine,
      Ikoma, Japan.
FAU - Miyata, Toshiyuki
AU  - Miyata T
AD  - Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular
      Center, Suita, Japan.
FAU - Murata, Mitsuru
AU  - Murata M
AD  - Department of Laboratory Medicine, Keio University School of Medicine, Tokyo,
      Japan.
CN  - For TTP group of Blood Coagulation Abnormalities Research Team, Research on Rare 
      and Intractable Disease supported by Health, Labour, and Welfare Sciences
      Research Grants
LA  - eng
PT  - Journal Article
DEP - 20170526
PL  - Japan
TA  - Int J Hematol
JT  - International journal of hematology
JID - 9111627
OTO - NOTNLM
OT  - ADAMTS13
OT  - TMA
OT  - TTP
EDAT- 2017/05/28 06:00
MHDA- 2017/05/28 06:00
CRDT- 2017/05/28 06:00
PHST- 2017/04/26 [received]
PHST- 2017/05/21 [accepted]
PHST- 2017/05/21 [revised]
AID - 10.1007/s12185-017-2264-7 [doi]
AID - 10.1007/s12185-017-2264-7 [pii]
PST - aheadofprint
SO  - Int J Hematol. 2017 May 26. doi: 10.1007/s12185-017-2264-7.

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