小児再生不良性貧血の長期予後

抄録

We studied long term prognosis of aplastic anemia in childhood. The patients have been registered and followed up by members of the Study Group of Hemopoietic Disorders sponsored by Ministry of Health and Welfare since 1973. The total 150 registered cases were composed of 118 cases of idiopathic aplastic anemia, 17 cases of secondary type, and 15 Fanconi anemias. The follow up period ranged from 1 day to 15 years and 5 months. These patients first visited to the member's clinic from September 1965 to June 1975. The survival rate was analysed with Kaplan-Meier method. The survival rate of well documented 93 patients with idiopathic aplastic anemia was 52.8±11.6% (SE). that of the secondary type was 30.9±24.8%, and none of the Fanconi were to survive longer than nine years. The outcome of the idiopathic aplastic anemia was well correlated with the initial hematological data. particularly with the absolute neutrophil counts, platelet counts, and bone marrow hemopoietic cell ratios. The Camitta's criterion was the best in predictibility for selection of the poorest prognosis group died within one year amongst the various classifications that have been currently proposed.