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Sir, Autoimmune haemolytic anaemia (AIHA) is a key feature of SLE. Most patients respond adequately to high-dose steroids (prednisolone 1 mg/kg) with gradual dose tapering. In ∼10% of patients AIHA is refractory or relapsing necessitating a second-line treatment [1]. In these cases, multiple agents have been tried including AZA, CYC, IVIG, ciclosporin, MMF, splenectomy and finally rituximab [2]. Herein we present the case of a 20-year-old female patient suffering from severe AIHA since the age of 7. Due to multiple relapses and short-lived responses to high-dose steroids, the patient underwent splenectomy at the age of 11 without significant benefit. At the age of 12 years, she received a first course of four weekly rituximab infusions (375 mg/m2 each) that resulted in complete remission for 1 year. Following a new relapse, a second course of rituximab was undertaken but had to be abandoned at the second infusion due to a severe allergic reaction requiring emergency resuscitation with epinephrine. Ever since, the patient has received high-dose steroids and IVIG as required for her haemolytic flares.
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Karageorgas et al. (2016) studied this question.
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