Occurrence of myeloproliferative disorder in patients with Noonan syndrome.

Bader-Meunier, B; Tchernia, G; Miélot, F; Fontaine, J L; Thomas, C; Lyonnet, S; Lavergne, J M; Dommergues, J P · J Pediatr · 1997

case_series · Level IV

Where this comes from

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.

Medical subject headings