Rapid transient reversal of anemia and long-term effects of maintenance intravenous immunoglobulin for autoimmune hemolytic anemia in patients with lymphoproliferative disorders.

Seven patients with autoimmune hemolytic anemia (AIHA) associated with an underlying lymphoproliferative disorder were treated with intravenous immunoglobulin. Five patients with chronic lymphocytic leukemia, a patient with Hodgkin's lymphoma with severe AIHA associated with a "warm" IgG antibody, and a patient with non-Hodgkin's lymphoma with an IgM "cold" antibody were treated with intravenous immunoglobulin G (0.4 g/kg) daily for five doses followed by maintenance therapy every 21 to 28 days if evidence of recurrence was noted. Two additional patients with refractory chronic lymphocytic leukemia and hypogammaglobulinemia were given maintenance therapy with intravenous immunoglobulin G every 21 days for previously recurrent AIHA and infections. Hematocrit levels of patients with AIHA stabilized followed by a gradual improvement at 21 days after intravenous immunoglobulin G infusion without steroids. Treatment with steroids and intravenous immunoglobulin G resulted in faster and higher increments in hematocrit levels in these patients. Other patients who had partial responses to steroids showed further improvement in their hematocrit levels by the addition of intravenous immunoglobulin G. Another patient with a cold agglutinin disease was refractory to intravenous immunoglobulin G therapy. Five patients with chronic lymphocytic leukemia and acute AIHA and two patients with previous recurrences of AIHA required maintenance intravenous immunoglobulin G every 21 days. All seven patients except one did not have any episodes of AIHA from six months to as long as four years while receiving the three-week intravenous immunoglobulin G therapy. These observations indicate a role for intravenous immunoglobulin G in the management of IgG-mediated but not IgM-associated autoimmune hemolysis in immunocompromised patients with lymphoproliferative diseases.