Occurrence of myeloproliferative disorder in patients with Noonan syndrome
Occurrence of myeloproliferative disorder in patients with Noonan syndrome
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DOI：
10.1016/s0022-3476(97)70273-7
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发表时间：
1997-06-01
影响因子：
5.1
通讯作者：
Dommergues, JP
中科院分区：
文献类型：
--
作者：
BaderMeunier, B;Tchernia, G;Dommergues, JP
本文报告4例儿童慢性粒单核细胞白血病合并努南综合征。这些儿童有一些共同的血液学特征：血小板减少，出生后最初几个月内脾肿大，发生慢性粒单核细胞白血病而无初始骨髓核型异常，其中3例血液病改善。
We report four cases of Noonan syndrome associated with chronic myelomonocytic leukemia in childhood, These children shared some hematologic features: thrombocytopenia, splenomegaly in the first months of life, occurrence of chronic myelomonocytic leukemia without abnormalities of the initial bone marrow karyotype, and, in three cases, improvement of the hematologic disease, A common pathophysiologic process in such patients is suggested.