A Case of Down Syndrome with Transient Abnormal Myelopoiesis and Progressive Liver Fibrosis.

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  • 進行性の肝線維化を伴ったｔｒａｎｓｉｅｎｔ　ａｂｎｏｒｍａｌ　ｍｙｅｌｏｐｏｉｅｓｉｓ（ＴＡＭ）併発Ｄｏｗｎ症候群の１例

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A female case of Down syndrome with transient abnormal myelopoiesis (TAM) associated with severe liver fibrosis is reported. Because of hyperviscosity syndrome secondary to leukocytosis at birth, she received mechanical ventilation, exchange transfusion, and chemotherapy. Herblast cells disappeared, and her general conditions get better through this management. However, her bilirubin level was maintained to elevate gradually. An abdominal CT scan revealed an atrophy of the right lobe of the liver and splenomegaly at 123 days of age. She died of hepatic failure at 145 days of age. The autopsy showed severe liver fibrosis without infiltration of blast cells. According to literature we investigated, all but one patient with Down syndrome complicated with TAM and liver fibrosis died within 100 days of age. TAM is generally considered to be a self-limiting disease. The prognosis, however, could be much worse if the syndrome is complicated with liver fibrosis. A further accumulation of data is needed to establish an effective therapy for a patient with TAM complicated with liver fibrosis.

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