title: Occurrence of myeloproliferative disorder in patients with Noonan syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Bicêtre Hospital, Le Kremlin Bicêtre, France.
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
authors.forename: F
authors.initials: F
authors.lastname: Miélot
authors.forename: J L
authors.initials: JL
authors.lastname: Fontaine
authors.forename: C
authors.initials: C
authors.lastname: Thomas
authors.forename: S
authors.initials: S
authors.lastname: Lyonnet
authors.forename: J M
authors.initials: JM
authors.lastname: Lavergne
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
mesh_terms: D001853:Bone Marrow / Q000737:chemistry; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007621:Karyotyping; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications* / Q000175:diagnosis; D008297:Male; D009634:Noonan Syndrome / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies; D013163:Splenomegaly / Q000188:drug therapy / Q000209:etiology; D013921:Thrombocytopenia / Q000175:diagnosis / Q000209:etiology