Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic anemia (AIHA) characterized by the presence of a Donath-Landsteiner (D-L) antibody. PCH occurs most commonly in young children and is associated with acute, often self-limited hemolytic anemia. The D-L antibody is classically a biphasic IgG anti-P autoantibody identified by the D-L test. Rare case reports confirm the existence of IgM D-L antibodies. We report the case of a 2-year-old male diagnosed with acute AIHA and found to have PCH caused by an IgA D-L antibody. The clinical course and treatment of this condition, which has not been reported previously, are described.
Warm-reactive IgM autoimmune hemolytic anemia is uncommon and carries a poor prognosis in adults. There have been rare reports in children, generally associated with an underlying immunologic deficiency, and outcomes are quite variable. Warm IgM in combination with other antibodies has not been reported in children. We report the first case of severe, steroid-responsive autoimmune hemolytic anemia caused by both warm-reactive IgM and IgA autoantibodies in an otherwise healthy 3-month-old.
Typing for antigens in the Dombrock blood group system and identifying the corresponding antibodies are notoriously difficult tasks. The reagents are scarce and the antibodies are weakly reactive. When RBCs from family members of a patient with an antibody to a high-prevalence Dombrock antigen were tested for compatibility, an unusual pattern of inheritance was observed: RBCs from the patient's children and one niece, in addition to those from some of the patient's siblings, were compatible. This prompted the performance of DNA-based assays for DO alleles and the results obtained were consistent with and explained the compatibility test results. It was possible to study this large kindred because of the cooperation of family members, hospital personnel, and reference laboratory staff.
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