抄録
A 64-year-old female was admitted to our hospital in March, 1976, for evaluation of severe anemia and leukopenia. Hematological data on admission were as follows: RBC 2.07×1012/l, Hb 6.6 g/dl, Ht 19.5%, MCV 94 fl, MCH 31.9 pg, MCHC 34.0 g/dl, reticulocyte 3.2%, WBC 2.2×109/l without abnormal cells, and platelets 200×109/l. Serial bone marrow examinations showed erythroid hyperplasia associated with slight increase of plasma cells, a few of which were polynuclear. The karyotype of bone marrow cells was normal. Haptoglobin levels were always remarkably low, but hemolysis test on the peripheral red blood cells were within normal limits. An immunological study revealed high level of IgA (λ). Treatment with prednisolone and vitamin B6 was disappointing for anemia and leukopenia. She gradually became pancytopenic and died of pulmonary infection in June, 1977. During her hospitalization there were no biochemical and hematological evidences indicative of sideroblastic anemia, multiple myeloma, or leukemia. The autopsy revealed hyperplastic marrow in sternal and femoral bones with slight extramedullary hematopoiesis in the spleen.
In this case a definite diagnosis was not made and the hematological improvement was not obtained. Though her hematological conditions were compatible with atypical aplastic anemia (Hasegawa), a diagnosis of refractory anemia with excess of blasts (Sultan) seems to be more suitable.
Pancytepenia with hyperplastic bone marrow and immunological abnormality in relation to hematopoietic dysplasia were discussed.
