A Case of Transient Abnormal Myelopoiesis with Myelofibrosis.
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A case of transient abnormal myelopoiesis (TAM) with myelofibrosis was reported. A 1-month-old male with Down's syndrome was admitted to our hospital because of hepatosplenomegaly and jaundice. The WBC count of peripheral blood increased to 69, 000/μl with 60% blasts, but neither anemia nor thrombocytopenia was recognized. Bone marrow examination showed the presence of 15% blasts cells. In cytochemical studies, a large number of blasts were peroxydase negative, naphtal AS-D chloroacetate esterase negative, α-naphthyl butyrate esterase negative and acid phosphatase positive. Fresh blood cells reacted to monoclonal antibodies and recognized platelet-associated antigen (pI-gpIIb/IIIa). The blast cells were positive for platelet peroxidase (PPO) on analysis by electron microscopy. We hypothesized that TAM evoked leukocytosis and no therapeutic measurement was applied. The blast cells gradually subsided in the peripheral blood. At that time, the bone marrow biopsy showed a marked fibrosis with no blast cells, and hepatic biopsy did not showed blast infiltration. The patient died when 60-days old because of hepatic complication. To establish the disease entity, further studies of many cases by cytochemical, immunological, and ultrastructural microscopic examination are necessary.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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