Juvenile myelomonocytic leukemia and Noonan syndrome
Juvenile myelomonocytic leukemia and Noonan syndrome
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DOI：
10.1097/00043426-199911000-00015
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发表时间：
1999-11-01
影响因子：
1.2
通讯作者：
Zipursky, A
中科院分区：
文献类型：
--
作者：
Choong, K;Freedman, MH;Zipursky, A
本文报告1例努南综合征（NS）新生儿的幼年粒单核细胞白血病（JMML，以前称为JCML）。该男孩表现为双侧先天性胸腔积液、非免疫性积液、畸形面容、持续性血小板减少和白细胞增多。JMML的诊断是在骨髓细胞培养研究中作出的。文献回顾显示，NS患者中血液系统恶性肿瘤，特别是JMML的异常优势。在作者所在机构10年期间收治的40例NS患者中，有4例（10%）有单核细胞增殖的证据，并自发消退。作者推测NS患者骨髓增生性疾病的发生率可能增加，在大多数情况下，骨髓增生性疾病似乎是良性的，但可能是白血病前期甚至是致命的。
A case of juvenile myelomonocytic leukemia (JMML, previously referred to as JCML) in a neonate with Noonan syndrome (NS) is described. The boy presented with bilateral congenital hydrothoraces, nonimmune hydrops, dysmorphic facies, persistent thrombocytopenia, and leukocytosis. The diagnosis of JMML was made on bone marrow cell culture studies. Review of the literature reveals an unusual preponderance of hematologic malignancies, in particular JMML, among patients with NS. Of 40 NS patients admitted to the authors' institution during a 10-year period, there were 4 (10%) with evidence of a monocytic proliferation, which resolved spontaneously. The authors postulate that patients with NS may have an increased incidence of myeloproliferative disorders, which in most cases appears to be benign but may be preleukemic or even lethal.