Mixed Autoimmune Hemolytic Anemia With Refractory Cold Agglutinin Syndrome in a Child Successfully Treated With Serial Whole Blood Exchange Transfusions and Immunosuppression

Abstract

Background: Mixed autoimmune hemolytic anemia can manifest with severe intra- and extravascular hemolysis, with autoagglutination contributing to vaso-occlusive microangiopathic phenomena.

Observations: We present a case of mixed autoimmune haemolytic anemia with unusually severe cold agglutinin syndrome causing cutaneous, cerebral, ocular, and aural manifestations of microvasculopathy and massive intravascular haemolysis, requiring 6 double-volume whole-blood exchanges over 8 days to temporise severe hemolysis and autoagglutination while immunosuppression with high-dose steroids, rituximab, and bortezomib took effect.

Conclusions: Whole-blood exchange permitted fluid-neutral clearance of preformed antibodies and antibody-coated red cells, with dramatic resolution of microvasculopathy and hemolysis, before discharge home on oral steroid therapy.

Keywords: cold agglutinin; hemolytic anemia; paediatric; whole blood exchange.