Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023.
ADAMTS13
TMA
TTP
Journal
International journal of hematology
ISSN: 1865-3774
Titre abrégé: Int J Hematol
Pays: Japan
ID NLM: 9111627
Informations de publication
Date de publication:
Nov 2023
Nov 2023
Historique:
received:
06
06
2023
accepted:
23
08
2023
revised:
21
08
2023
medline:
2
11
2023
pubmed:
10
9
2023
entrez:
9
9
2023
Statut:
ppublish
Résumé
Thrombotic thrombocytopenic purpura (TTP) can rapidly become a life-threatening condition, and the importance of its appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and 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 become internationally accepted as a diagnostic criterion for TTP. TTP is classified as immune-mediated TTP (iTTP) if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital TTP (cTTP) if ADAMTS13 gene abnormalities are detected. Fresh frozen plasma (FFP) transfusion is performed in patients with cTTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with iTTP to supplement ADAMTS13 and to remove both anti-ADAMTS13 autoantibodies and unusually large von Willebrand factor (VWF) multimers. To suppress autoantibody production, corticosteroid therapy is administered in conjunction with plasma exchange. The monoclonal anti-CD-20 antibody rituximab is effective in patients with iTTP. In addition, caplacizumab, an anti-VWF A1 domain nanobody, has a novel mechanism of action, involving direct inhibition of platelet glycoprotein Ib-VWF binding. The recommended first-line treatments of iTTP in Japan are plasma exchange and corticosteroids, as well as caplacizumab.
Identifiants
pubmed: 37689812
doi: 10.1007/s12185-023-03657-0
pii: 10.1007/s12185-023-03657-0
pmc: PMC10615956
doi:
Substances chimiques
von Willebrand Factor
0
Autoantibodies
0
ADAMTS13 Protein
EC 3.4.24.87
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
529-546Subventions
Organisme : Ministry of Health, Labour and Welfare
ID : 20FC1024
Informations de copyright
© 2023. The Author(s).
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