Successful control of refractory and life-threatening autoimmune hemolytic anemia with intravenous immunoglobulins in a man with the primary antiphospholipid syndrome.

A 58-year old man with a history of hypothyroidism and primary antiphospholipid syndrome (with recurrent thromboembolic disease and therapy-refractory autoimmune thrombocytopenic purpura) presented with a life-threatening crisis of warm autoimmune hemolytic anemia (AIHA) while under chronic low-dose steroid therapy. The exacerbation was eventually controlled with a 5-day course of intravenous immunoglobulin (IVIG, Sandoglobulin) (400 mg/kg per day) but hemolysis rapidly recurred, despite therapy with steroids, azathioprine, and cyclosporin, necessitating a second course of IVIG. Control of packed cell transfusion needs for about 7 months was achieved by weekly administration of IVIG (800 mg/kg), although there is no direct evidence that IVIG therapy reduced the production of anticardiolipin or RBC antibodies. Three months after discontinuation of IVIG and change to maintenance with intermediate-dose corticosteroids plus cyclosporin A, the patient succumbed to duodenal perforation with peritonitis and invasive pulmonary aspergillosis. The case illustrates that IVIG therapy may be helpful in selected life-threatening and refractory cases of AIHA. It also sadly illustrates the long-term toxicity of standardly used therapeutics in refractory AIHA.