Occurrence of myeloproliferative disorder in patients with Noonan syndrome.
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.
Bader-Meunier
B
B
Department of Pediatrics, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Tchernia
G
G
Miélot
F
F
Fontaine
J L
JL
Thomas
C
C
Lyonnet
S
S
Lavergne
J M
JM
Dommergues
J P
JP
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