Rituximab prolongs the time to relapse in patients with immune thrombotic thrombocytopenic purpura: analysis of off-label use in Japan.
ADAMTS13 Protein
/ deficiency
Adult
Aged
Autoantibodies
Disease-Free Survival
Female
Follow-Up Studies
Humans
Japan
Male
Middle Aged
Off-Label Use
Purpura, Thrombocytopenic, Idiopathic
/ drug therapy
Recurrence
Retrospective Studies
Rituximab
/ therapeutic use
Secondary Prevention
Time Factors
Treatment Outcome
Immune TTP
Relapse
Rituximab
Journal
International journal of hematology
ISSN: 1865-3774
Titre abrégé: Int J Hematol
Pays: Japan
ID NLM: 9111627
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
01
07
2020
accepted:
17
08
2020
revised:
06
08
2020
pubmed:
29
8
2020
medline:
26
11
2020
entrez:
29
8
2020
Statut:
ppublish
Résumé
Immune thrombotic thrombocytopenic purpura (iTTP) is caused by ADAMTS13 deficiency due to anti-ADAMTS13 autoantibodies. Rituximab, an anti-CD20 monoclonal antibody, is often used to suppress these autoantibodies. This retrospective study, conducted in an iTTP cohort in Japan, evaluated the long-term efficacy of rituximab as off-label treatment for refractory or relapsed cases. A total of 252 iTTP patients with severe ADAMTS13 deficiency (< 10%) and its inhibitor were enrolled, and 169 episodes in 156 patients were analyzed. Sixty-five episodes with relapse or resistance to conventional treatment were treated with rituximab, while 104 episodes received conventional treatment only. The rituximab group had a significantly higher inhibitor titer than the rituximab-untreated group. During the median follow-up period of 3.9 years, there were 8 relapses in the rituximab group and 17 relapses in the rituximab-untreated group. The median time to relapse in the rituximab group (2.9 years) was significantly longer than that in the rituximab-untreated group (1.2 years). Relapse-free survival at 2 years was significantly higher in the rituximab group than in the rituximab-untreated group. The incidence of relapse at 5 years did not differ between the 2 groups. Rituximab reduced the risk of relapse in refractory or relapsed iTTP patients for 2 years.
Identifiants
pubmed: 32856231
doi: 10.1007/s12185-020-02974-y
pii: 10.1007/s12185-020-02974-y
doi:
Substances chimiques
Autoantibodies
0
Rituximab
4F4X42SYQ6
ADAMTS13 Protein
EC 3.4.24.87
ADAMTS13 protein, human
EC 3.4.24.87
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
764-772Références
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