Favorable and Sustained Response of Primary Autoimmune Hemolytic Anemia to Rituximab

Primary autoimmune hemolytic anemia (PAIHA) is a rare autoimmune disorder that males and females of all age groups, but more frequent in young adults. Most patients respond initially to steroids and intravenous immunoglobulin's followed by Splenectomy for non-responders. Around one third of the patients do not respond to any standard treatment and another one third show only transient response and therefore labeled as refractory PAIHA. In this refractory group, various immune modulators and suppressants are used with variable response. These include azathioprine, vincristine, cyclophosphamide and mycophenolate.

The aim of our study is to explore the safety and efficacy of Rituximab in the treatment of refractory, PAIHA.

Thirteen patients (seven males and six females, aged 33. 7 +/− 15 years) with refractory PAIHA (Hb 73. 1 +/− 13. 9 g/l) were given Rituximab 375 mg/m2 intravenously weekly for four weeks, then monthly for four months.

All patients showed a rapid and favorable response with significant rise in Hb level to 128 +/− 10. 8 g/l within four months. Rituximab was tolerated very well and the response was sustained in all patients during the follow up to 25. 6 +/− 21. 8 months

Rituximab is probably highly effective in the treatment of refractory PAIHA with sustainable response. This has been shown in refractory autoimmune hemolytic anemia in adults and children as well as in chronic lymphocytic leukemia associated autoimmune hemolytic anemia (1–3)

Off Label Use: Rituximab was used in Primary Autoimmune Hemolytic Anemia compassionately.