1987 Volume 28 Issue 4 Pages 616-623
Hemopoietic dysplasia in childhood is different from myelodysplastic syndrome in adults. A number of atypical cases that could not be categorized to the any classical entity have been registrated to the Study Group of Hemopoietic Disorders sponso ed by Ministry of Health and Welfare. In this paper, we presented 5 children with aplastic anemia who showed prodromal course of pre-aplastic state. Case 1: A six-year-old boy was presented with pancytopenia accompanied with normocellular marrow. The bone marrow smear revealed the increase of atypical dense granulated promyelocytes. After the treatment with vincristine and prednisolone, the patient had been in hematological remission for 3 years and 9 months, maintained with 6MP. Immediately after the discontinuation of 6MP, he developed pancytopenia with hypocellular marrow. At this time there was no increase of the atypical cells. The pancytopenia has been persisted for 3 years thereafter. Case 2: A four-year-old girl was presented with thrombocytopenia and neutropenia with normocellular marrow. Morphological abnormalities in erythroid and myeloid cells were observed. She was treated with meptiostane under the diagnosis of pre-aplastic state and the anemia was improved. Following the cessation of the therapy, however, the patient showed typical clinical manifestation of aplastic anemia. Case 3: A six-year-old girl was presented with thrombocytopenia but not anemia. Her marrow cellularity was diverse and megakaryocytes were decreased. Her clinical course has been followed without treatment. Case 4: A six year-old girl was presented with thrombocytopenia accompanied with normocellular marrow. She was treated with prednisolone under the diagnosis of chronic idiopathic thrombocytopenic purpura. Four months later, she developed typical aplastic anemia. Case 5: A four-year-old boy was presented with anemia and thrombocytopenia accompanied with hypocellular marrow. His peripheral leukocyte and neutrophil counts were normal. There were internuclear chromatin bridge of the erythroblasts and hypersegmentation of the myeloid cells. To confirm diagnosis of atypical cases, comprehensive assessment including analysis of ferrokinetics, hemopoietic stem cell culture and bone marrow biopsy were important and helpful. We suggest that there are some cases with features of hemopoietic dysplasia that may eventually develop aplastic anemia in childhood.
