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

For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants

Research output: Contribution to journalArticle

7 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1-13
Number of pages13
JournalInternational Journal of Hematology
DOIs
Publication statusAccepted/In press - 2017 May 26

Fingerprint

Thrombospondin 1
Disintegrins
Thrombotic Thrombocytopenic Purpura
Metalloproteases
Japan
Guidelines
Autoantibodies
Plasma Exchange
Therapeutics
Idiopathic Thrombocytopenic Purpura
Hemolytic Anemia
Adrenal Cortex Hormones
Antibodies
Genes

Keywords

  • ADAMTS13
  • TMA
  • TTP

ASJC Scopus subject areas

  • Hematology

Cite this

For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants (Accepted/In press). Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. International Journal of Hematology, 1-13. https://doi.org/10.1007/s12185-017-2264-7

Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. / For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants.

In: International Journal of Hematology, 26.05.2017, p. 1-13.

Research output: Contribution to journalArticle

For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants 2017, 'Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan', International Journal of Hematology, pp. 1-13. https://doi.org/10.1007/s12185-017-2264-7
For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. International Journal of Hematology. 2017 May 26;1-13. https://doi.org/10.1007/s12185-017-2264-7
For Ttp Group Of Blood Coagulation Abnormalities Research Team, Research On Rare And Intractable Disease Supported By Health, Labour, And Welfare Sciences Research Grants. / Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. In: International Journal of Hematology. 2017 ; pp. 1-13.
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