Evaluation of a patient prompted the systematic review of causes of abrupt onset
anemia in children and the development of a protocol for the immunohematologic workup of such cases. The patient, a 3- year-old boy, presented to our institution two weeks following a viral illness with gross
hematuria,
jaundice, and severe
anemia (hematocrit 14.5%). He was found to have a positive direct antiglobulin test (DAT) due to primarily
complement sensitization (2+) with smaller amounts of
IgG (weak). A nonspecific cold
agglutinin, anti-P1, and a hemolytic biphasic
Donath-Landsteiner antibody were demonstrated in his serum, and he was diagnosed with
paroxysmal cold hemoglobinuria (PCH). The
hemolytic anemia resolved with packed red cell transfusions,
intravenous immune globulin, and
steroid treatment. It was concluded that a complete immunohematologic evaluation, including a DAT, cold
agglutinin, and
Donath-Landsteiner antibody tests, is pivotal to prompt and accurate assessment of the cause of an abrupt onset of severe
anemia in children.