Occurrence of myeloproliferative disorder in patients with Noonan syndrome

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 m...

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Veröffentlicht in:The Journal of pediatrics 1997-06, Vol.130 (6), p.885-889
Hauptverfasser: Bader-Meunier, B., Tchernia, G., Miélot, F., Fontaine, J.L., Thomas, C., Lyonnet, S., Lavergne, J.M., Dommergues, J.P.
Format: Artikel
Sprache:eng
Schlagworte:
Biological and medical sciences
Bone Marrow - chemistry
Diseases of the osteoarticular system
Female
Humans
Infant
Infant, Newborn
Karyotyping
Leukemia, Myelomonocytic, Chronic - complications
Leukemia, Myelomonocytic, Chronic - diagnosis
Male
Medical sciences
Noonan Syndrome - complications
Noonan Syndrome - diagnosis
Retrospective Studies
Splenomegaly - drug therapy
Splenomegaly - etiology
Thrombocytopenia - diagnosis
Thrombocytopenia - etiology
Vascular bone diseases
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Zusammenfassung: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. (J Pediatr 1997; 130:885-9)
ISSN:0022-3476
1097-6833
DOI:10.1016/S0022-3476(97)70273-7