CONGENITAL HYPOPLASTIC ANEMIA

[摘要] Peripheral blood values and the results of bone marrow studies in 12 children suffering from congenital hypoplastic anemia are presented. In these cases the erythrocyte counts were more than 1,000,000 cells below normal, the values for hemoglobin were more than 1 gm. below normal, and the total leukocyte counts were within the range of normal except in two cases. The differential leukocyte counts showed lymphocytosis (50 to 90.5%). The mean percentage for reticulocytes was 0.3, and the platelet counts ranged from 71,000 to 409,000, except in two instances.Studies of bone marrow concentrations in six children showed a decreased myeloiderythroid layer. In 10 children differential counts of 1,000 nucleated cells in the marrow showed decrease of the percentages for normoblasts, with a range from 1.2 to 13.8 and a mean of 3.7. Myelopoiesis was fairly active, percentages for myeloid cells ranging from 32.5 to 63.4 with a mean of 46.4. The percentages for lymphocytes were increased with a range from 30.6 to 63.6 and a mean of 48.5. Maturation of the normoblasts blasts was arrested at the polychromatic stage. The myeloid-erythroid ratio was greatly increased, with a range from 4:1 to 43:1 and a mean of 19:1.In the 12 children of this series the onset was before 15 months of age. There was a familial incidence of the disease in three cases. The anemia recurred every 3 to 8 weeks as long as the hypoplasia persisted, and transfusions provided the only means of controlling the anemic state. Nevertheless, most of these children grew, developed normally, and carried on normal activities. Sponstaneous remissions occurred in five. The duration of these remissions at the time of the last reports ranged from 20 months to approximately 8 years.

[学科分类] 儿科学