A case of childhood autoimmune hemolytic anemia (AIHA) combined with idiopathic thrombocytopenic purpura (ITP) is reported. This clinical condition, known as “Evans' syndrome”, was associated with plural positive serologic data for systemic lupus erythematosus (SLE) with abscence of the physical signs proposed by American Rheumatism Association (ARA). The autoantibody on the patient's red blood cells was identified as panreactive IgG which contained both kappa and lambda light chains, but antiplatelet antibody was not detected. Pulse mode therapy using methylprednisolone was instituted in acute phase and was effective without side effects of steroid hormone. Although the urinalysis had been unremarkable, the percutaneous renal biopsy performed five-month later revealed the histopathological change compatible with proliferative glomerulonephritis. Both AIHA and ITP seem to have autoimmune characters and their pathogenetic mechanisms are reviewed. Diagnostic consideration of subclinical SLE is also described.
