Rituximab is becoming the preferred second-line therapy for wAIHA, with an 80% overall response rate, a median response time of 3 to 6 weeks (with a range of 2 to 16 weeks), and a sustained response rate of 60% at 3 years.
It has been thoroughly researched and has proven effective in primary and most secondary forms of wAIHA, and retreatment appears similarly successful.
Various doses have been used
(375 mg/m2 per week for 4 weeks, most commonly) 100 mg weekly for 4 weeks, mainly in nonsevere forms and in the elderly
1 g on days 1 and 15, particularly in wAIHA associated with other autoimmune diseases), with quite similar efficacy (response rate ;80%,relapse-free survival of 60% at 3 years), although direct comparisons have not been made.
An interesting approach
is to use rituximab in first-line therapy:in a prospective randomized trial, rituximab with prednisolone increased the rate and duration of the response compared with prednisolone alone.
Furthermore, a randomized double-blind trial demonstrated the efficacy and safety of rituximab vs placebo among newly diagnosed patients on standard prednisone therapy.
However, rituximab is not available worldwide, nor is it universally available from public health services. Moreover, its significant side effects must be considered, particularly immunodeficiency (lateonset neutropenia, hypogammaglobulinemia) and reactivation of underlying infections (hepatitis B [HB] virus [HBV], hepatitis C [HCV], HIV, tuberculosis). Various tests (HB surface antigen [HBsAg], antiHBs, anti-HBc, anti-HCV, anti-HIV, and quantiFERON-TB) are advisable before steroid therapy, but mandatory before rituximab .
Lamivudine prophylaxis for up to 18 months is recommended for anti-HBc and/or anti-HBsantibody-positive patients (if not vaccinated).
Splenectomy is as effective as rituximab, with somewhat longer lasting remissions . Use of splenectomy has gradually declined,
mainly due to the increased infection risk, particularly in the first year. This risk is not eliminated by vaccination and antibiotic prophylaxis. Notably, vaccination may result in a suboptimal response after immunosuppression, and is therefore recommended before commencing rituximab, in line with national guidelines.
Reference
ASH
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