A 2-year-old male presented with upper respiratory tract infection sym
ptoms, continuous high fever, extensive truncal rash with desquamation
, lymphadenopathy, subconjunctival hemorrhage, and oral stomatitis. He
was diagnosed with Kawasaki disease and did well on aspirin. Approxim
ately 8 weeks after initial presentation he had evidence of severe imm
une hemolysis. At that time a direct antiglobulin test was microscopic
ally positive; it became strongly positive (3 + IgG, w + C3) 2 weeks l
ater. The serology was unusual in that a warm IgG autoantibody and a l
ow titer high thermal range cold antibody of unusual specificity (anti
-En(a) or anti-Pr) were present. We were uncertain as to which antibod
y caused the hemolysis, or whether they worked synergistically. The he
molysis resolved following treatment with high dose prednisone.