青少年型骨髓单核细胞白血病

定义 英文原文（暂无中文）

A rare myelodysplastic/myeloproliferative neoplasm characterized by a proliferation primarily of granulocytic and monocytic lineages with infiltration of the liver and spleen, among other organs. Blasts and promonocytes account for less than 20% of white blood cells in peripheral blood and bone marrow. Erythroid and megakaryocytic abnormalities are often present. BCR-ABL1 fusion is absent, while somatic mutations in genes of the RAS pathway or monosomy 7 may be found. The condition may also occur in the context of neurofibromatosis type 1 or Noonan syndrome-like disorder. Children of less than three years are predominantly affected, with a clear male preponderance. Most patients present with constitutional symptoms, signs of infection, and hepatosplenomegaly.

别名

青少年型慢性粒-单核细胞白血病

基本事实

遗传方式

不适用

发病年龄

儿童期、婴儿期

患病率

1-9 / 1 000 000（Europe）

相关基因 6

外部标识与链接