Few therapeutic options are available for severe, life threatening refractory cases of autoimmune hemolytic anemia (AIHA). We report a 53-year-old 110 kg male with acute onset of symptomatic severe anemia with syncope, unstable angina and jaundice. His nadir hematocrit was 8.3 vol% with peak total bilirubin of 44 mg/dL. Thirteen years previously he had undergone splenectomy for idiopathic thrombocytopenic purpura without recurrence of thrombocytopenia. The direct antiglubulin test (DAT) was positive but the indirect was negative. Elution studies demonstrated an IgG pan-agglutinin reactive at 37°C. Medical treatment with high dose corticosteroids and IVIg was instituted. An accessory spleen measuring 2 cm was identified and surgically removed on day 12 but he continued to have intense hemolysis. Cyclophosphamide at 200 mg per day was started on day 15. Apheresis with a Staphylococcal protein A immunoadsorption column (Prosorba(r)) was initiated on day 18. 1,250 ml of plasma were processed twice weekly for a total of six treatments. Cyclophosphamide was continued for a total of 14 days. His transfusion requirement ceased by the third immunoadsorption treatment. Forty units of red blood cells had been required over 23 days in an attempt to maintain a hematocrit >15 vol%. His DAT became negative after his fourth treatment. His hematocrit normalized at 44.9 vol% with bilirubin of 0.3 mg/dL three weeks after completion of the final protein A immunoadsorption treatment. Prednisone was tapered and discontinued. This complete response has remained durable for more than six months. Our results suggest that Staphylococcal protein A immunoadsorption may be an effective treatment in severe refractory warm AIHA. (Figure Presented).
|Original language||English (US)|
|Issue number||11 PART II|
|State||Published - Dec 1 2000|
All Science Journal Classification (ASJC) codes
- Cell Biology