We report a case of Evans syndrome with pregnancy-induced elevation of macrophagecolony stimulating factor (M-CSF). A 26-year-old woman presented in May 1989 because of slight dizziness and dyspnea. Peripheral blood analysis revealed RBC 224×10⁴/μl, Hb 7.8g/dl, Ht 23.8%, Ret 5.1%, WBC 9, 500/μl, Plt 23.5×10⁴/μl, LDH 777U/l, total bilirubin 1.0mg/dl, and haptoglobin <10mg/dl. A direct Coombs test was positive and IgG autoantibody was identified. The patient was diagnosed as having the idiopathic warm antibody type of autoimmune hemolytic anemia (AIHA), and therapy was started with prednisolone 30mg/day. The anemia improved during steroid therapy. The patient delivered her first child in December 1989. In April 1991, she had both AIHA and idiopathic thrombocytopenic purpura (ITP), and was diagnosed as having Evans syndrome. After her second delivery, she underwent γ-globulin therapy and splenectomy. In March 1995, she delivered her third child, without progression of AIHA or ITP. Except for during the third pregnancy, which occurred after splenectomy, gestation was associated with worsened AIHA and ITP as well as elevation of M-CSF. These findings suggest that the increase in M-CSF associated with pregnancy could contribute to the pathology of the patient in this report.

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