Recurrent cold hemagglutinin disease following allogeneic bone marrow transplantation successfully treated with plasmapheresis, corticosteroid and cyclophosphamide.

A 10-year-old male with severe aplastic anemia following allogeneic BMT developed a hemolytic crisis on post-BMT day 67. The diagnosis of cold hemagglutinin disease was made based on the findings of anemia, reticulocytosis, positive direct Coombs test, and markedly elevated cold agglutinins. Anti-nuclear antibody and anti-DNA antibody were also increased. Plasmapheresis was effective as an emergency measure. Corticosteroid after plasmapheresis had a transient effect. At the second episode of hemolysis 6 months after BMT, immunosuppressive therapy with cyclophosphamide plus corticosteroid was successfully administered without negative effect on engraftment.