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. 1997 Jun;130(6):885-9.
doi: 10.1016/s0022-3476(97)70273-7.

Occurrence of myeloproliferative disorder in patients with Noonan syndrome

B Bader-Meunier  1 , G Tchernia, F Miélot, J L Fontaine, C Thomas, S Lyonnet, J M Lavergne, J P Dommergues
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Occurrence of myeloproliferative disorder in patients with Noonan syndrome

B Bader-Meunier et al. J Pediatr. 1997 Jun.

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.

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