Autoimmune haemolytic anaemia (AIHA) is an uncommon condition characterized by increased destruction of erythrocytes associated with reticulocytosis in the great majority of cases. We present the case of a 68-year-old woman with jaundice and malaise. Investigation revealed AIHA with reticulocytopenia. The patient failed to respond to prednisolone or to rituximab. Azathioprine and epoetin beta were subsequently started, the prednisolone dose was increased, and the patient began to respond after 1 month. In AIHA, reticulocytopenia is a very rare presentation and a sign of great severity and poor outcome. The scarcity of therapeutic options in refractory cases poses a major challenge for physicians. CONCLUSIONS Autoimmune haemolytic anaemia is a rare disorder characterized by decompensated acquired haemolysis caused by the host's immune system acting against its own red cell antigens.Concomitant presentation with reticulocytopenia is very rare and a sign of great severity and poor outcome.Treatment options in refractory cases still greatly rely on individual experience and expert opinion.
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