Efficacy of bortezomib in non-IgM type I cryoglobulinaemic vasculitis: a single-centre retrospective case series.
Eur J Haematol
; 2018 Jul 30.
Article
em En
| MEDLINE
| ID: mdl-30058297
ABSTRACT
INTRODUCTION:
Treatment of non-IgM type I cryoglobulinaemic vasculitis (CV) is challenging. Corticosteroids are first-line therapy, but relapses are frequent leading to therapeutic escalation. Bortezomib is a proteasome inhibitor with rapid effect on monoclonal component. However, its use in non-IgM type I CV has been barely reported.OBJECTIVE:
To assess the efficacy of bortezomib in non-IgM type I CV.METHOD:
Single-centre case series of four patients with non-IgM type I CV treated with bortezomib monotherapy.RESULTS:
Two men and two women, 60-84 years old, received bortezomib monotherapy. Monoclonal component was IgG-λ (n = 2), IgA-λ and IgG-κ. Clinical features were necrotic rash (n = 3), synovitis (n = 3) and sensitive neuropathy (n = 2). CV was refractory to corticosteroids (n = 4), cyclophosphamide (n = 3) and rituximab (n = 2). Three patients experienced dramatic clinical improvement with undetectable cryoglobulin after three cycles (bortezomib 1.3 mg/m2 weekly). Each patient relapsed 4-18 months after treatment discontinuation. Bortezomib was unsuccessful after four cycles in one patient. Bortezomib toxicity included one pneumonia and 1 case of worsening neuropathic pain.CONCLUSION:
Bortezomib in monotherapy should be considered as a valuable option in refractory non-IgM type I CV because of its swift efficacy and acceptable tolerance profile.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Idioma:
En
Revista:
Eur J Haematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
França