7. 自己免疫性溶血性貧血の治療：現況と問題点

抄録

The current status of the therapeutic aspects and long-term clinical course of warm-type autoimmune hemolytic anemia (AIHA) was summarized. The data were derived from the patient materials which had been collected under cooperation of many major institutions and clinics by the Research Committee of Hemolytic Anemias (Chairman: Prof. Shiro Miwa, 1974∼1976) and that of Idiopathic Hematopoietic Disorders (Chairman: Haruto Uchino, 1977∼1982), the Ministry of Public Health and Welfare.
A total of 185 patients (152 with idiopathic and 33 with symptomatic variety) was followed up for the respective average periods of 7.4 and 4.5 years. The overall mortality during these periods was 28% for idiopathic and 58% for symptomatic AIHA. The survival fraction of the former declined steadily until an apparent plateau was reached at 65% after 10 years from onset. This indicates that potentially serious problems distribute over the wide time span ranging from onset to the order of at least 10 years. After prolonged clinical course (mean: 9.4 years), 43% of patients with idiopathic AIHA were off treatment and the rest were kept under the continued maintenance therapy, of which 67% was with minimal doses of corticosteroid only and 17% with a combined use of cytotoxic immunosuppressive agents. Irrespective of maintenance treatment, a majority of patients (84%) ran a fairly stable clinical course. Splenectomy was performed in 20 patients (13.2%). Roughly 10% of patients were presumed to be in the state of an apparent clinical cure.
The preliminary results from the separately performed prospective study on 47 cases with idiopathic AIHA revealed excellent or favorable response to initial treatment with 1∼1.5 mg/kg prednisolone in 93% of patients. The hematological remission was achieved by 4 weeks in 50% and by 2 months in 83% of patients.
Based on these data, the long-term evolution and natural history of AIHA were discussed.