抄録
Refractory anemia with excess of blasts (RAEB) and erythroleukemia (M6) occured in two brothers are reported.
An elder brother, 22-year-old man was admitted to Kyushu University Hospital in November 1985 because of anemia. The white cell count was 8,900/μl with 5% myeloblasts. The hemoglobin was 11.2g/dl and the platelet count was 42,000/μl. Microscopical examination of an aspirated specimen of bone marrow revealed normal cellularity with 6% myeloblasts and dysplastic changes in three lineages of hematopoietic cells. The chromosomal analysis of bone marrow cells showed multiple cytogenetic abnormalities. A diagnosis of RAEB was made. The patient has been followed for a year and a half but any hematological change has not been observed. His younger brother, 20-year-old man was admitted to Hamanomachi Hospital in July 1985 because of general fatigue. The white cell count was 2,600/μl with 36% myeloblasts. Hemoglobin was 5.2g/dl and the platelet count was 33,000/μl. Microscopical examination of an aspirated specimen of bone marrow revealed normal cellularity with 62.8% abnormal erythroblasts and 25.2% myeloblasts. A diagnosis of erythroleukemia (M6) was made. Antileukemic agents (DCMP therapy) were administrated, but complete remission was not achieved. He died because of cerebral hemorrhage in May 1986. Although the clinical courses of the two brothers were different, the similarity of dyshematopoietic findings suggests the presence of common genetic disorder in the pluripotent hematopoietic stem cells.
