1003 SUCCESSFUL TREATMENT OF AUTOIMMUNE HEMOLYTIC ANEMIA (AIHA) IN COMMON VARIABLE IMMUNODEFICIENCY (CVID) WITH HIGH-DOSE INTRAVENOUS IMMUNE SLOBULIN (IVIS)

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Standard therapy for AIHA with steroids, cytotoxic agents and/or splenectomy carries an even greater risk than in normals for patients with primary immunodeficiency. Because of the success of high dose IVIG therapy in immune thrombocytopenia, we treated a male with CVID and AIHA with large doses of a pH 4.0 IVIG preparation as a potentially effective means of therapy. A diagnosis of CVID (IgG 330, IgA 4, IgM 24 mg/dl) had been made at age 18 years following a history of chronic sinopulmonary disease and splenomegaly, and he was begun on I.M. gamma globulin at 100 mg/kg/mo at that time. He had no hematologic abnormalities until age 21 when he presented with acute AIHA (hgb 5.3 g/dl, haptoglobin <5 mg/dl and a 4 + positive direct Coombs). After 60 mg/d of prednisone for 2 months, his hgb rose to 12.8 and his haptoglobin to 50 mg/dl. However, his Coombs test remained 4 + positive for IgG antibody and his retic ct. was 9.7. Steroids were discontinued and therapy with 450 mg/kg IVIG was initiated and continued daily for 5 days; He was then given 100-200 mg/kg of IVIG at 4 week intervals. During this period, his hgb ranged from 12.5-14 g/dl and his retic ct. dropped to 0.5-2.3. After 7 months of IVIG therapy, his hgb is 12.6 g/dl, retic ct. 1.6%, haptoglobin 147 mg/dl and Coombs test negative. These observations provide strong support for the use of high dose IVIG in immunodeficient patients with AIHA. It is safe and a mere modification of standard replacement therapy for humoral immunodeficiency.