努南综合征患者骨髓增殖性疾病的发生情况。
Occurrence of myeloproliferative disorder in patients with Noonan syndrome.
作者信息
Bader-Meunier B, Tchernia G, Miélot F, Fontaine J L, Thomas C, Lyonnet S, Lavergne J M, Dommergues J P
机构信息
Department of Pediatrics, Bicêtre Hospital, Le Kremlin Bicêtre, France.
出版信息
J Pediatr. 1997 Jun;130(6):885-9. doi: 10.1016/s0022-3476(97)70273-7.
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.
我们报告了4例儿童期与慢性粒单核细胞白血病相关的努南综合征病例。这些儿童有一些共同的血液学特征：血小板减少、出生后最初几个月脾肿大、发生慢性粒单核细胞白血病且初始骨髓核型无异常，3例患者血液系统疾病有所改善。提示这类患者存在共同的病理生理过程。
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