Intravascular hemolysis by IgA red cell autoantibodies.
A 66-year-old male patient with severe intravascular hemolysis is presented. Laboratory investigation revealed initially a negative direct antiglobulin test (DAT), suggesting a Coombs-negative hemolytic anemia. Additional testing with monospecific anti-IgA was strongly positive. IgA autoantibodies with anti-e specificity and nonspecific IgA autoantibodies were identified. A diagnosis of IgA-only-associated warm AIHA was made. Treatment included transfusion of multiple e-negative typed red cell concentrates and administration of high-dose prednisone. The pathophysiologic mechanism of the rare IgA-induced warm AIHA is discussed.
Beckers
E A
EA
Bloodbank Rotterdam, Wytemaweg 10, 3015 CN, Rotterdam, The Netherlands. erik.beckers@bloodrtd.nl
van Guldener
C
C
Overbeeke
M A
MA
van Rhenen
D J
DJ
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D000888:Antibodies, Anti-Idiotypic / Q000032:analysis / Q000502:physiology*; D001323:Autoantibodies / Q000032:analysis / Q000502:physiology*; D003298:Coombs Test; D006461:Hemolysis; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D008297:Male
D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D007070:Immunoglobulin A