Juvenile myelomonocytic leukaemia and Noonan syndrome

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Juvenile myelomonocytic leukaemia and Noonan syndrome

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Juvenile myelomonocytic leukemia and Noonan syndrome.

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 prepon...

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The mutational spectrum of PTPN11 in juvenile myelomonocytic leukemia and Noonan syndrome/myeloproliferative disease.

Germ line PTPN11 mutations cause 50% of cases of Noonan syndrome (NS). Somatic mutations in PTPN11 occur in 35% of patients with de novo, nonsyndromic juvenile myelomonocytic leukemia (JMML). Myeloproliferative disorders (MPDs), either transient or more fulminant forms, can also occur in infants with NS (NS/MPD). We identified PTPN11 mutations in blood or bone marrow specimens from 77 newly rep...

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 2014

ISSN: 0022-2593,1468-6244

DOI: 10.1136/jmedgenet-2014-102611