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[一名患有唐氏综合征和肝硬化的新生儿的单核母细胞型短暂异常骨髓造血]

[Transitory abnormal myelopoiesis of the monoblastic form in a newborn infant with Down's syndrome and liver cirrhosis].

作者信息

Urano Y, Kondo M, Hayashi Y, Wakuya J, Nakata T, Tanaka K, Seo A

出版信息

Ann Pathol. 1983 Dec;3(4):337-9.

PMID:6230084
Abstract

A case of 5-day old newborn infant with trisomy 21, who presented a leucocytosis (151 000/ml) with 60% of monoblasts and marked hepatosplenomegaly is reported. Transient abnormal myelopoiesis (TAM) was retained. TAM regressed spontaneously. However the baby died at the age of 50 days. The autopsy showed extramedullary hematopoiesis with marked monocytosis and liver cirrhosis of neonatal hepatitic origin. The pathogenesis of TAM in this case was discussed.

摘要

报告了一例患有21-三体综合征的5日龄新生儿,其白细胞增多(151000/ml),原始单核细胞占60%,并有明显肝脾肿大。诊断为短暂异常髓系造血(TAM)。TAM自行消退。然而,该婴儿在50日龄时死亡。尸检显示存在髓外造血,伴有明显的单核细胞增多以及新生儿肝炎所致的肝硬化。讨论了该病例中TAM的发病机制。

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