Transient abnormal myelopoiesis in Noonan syndrome.
An infant affected by Noonan syndrome and presenting with abnormal hemopoiesis is described. Cytogenetic analysis, molecular studies, and in vitro culture assays ruled out myelodysplasia or other hematologic malignancies but were consistent with transient abnormal myelopoiesis. At present, 3 years and 10 months after diagnosis, the patient shows a normal hematologic picture. Supportive treatment in such patients, unless refractory cytopenias or overt leukemia develops, is emphasized.
Silvio
Ferraris
F
Department of Pediatrics, University of Turin, Piazza Polonia 94, 10126 Turin, Italy. cnr@pediatria.unito.it
Carlo
Lanza
L
Elena
Barisone
B
Nicoletta
Bertorello
B
Daniela
Farinasso
F
Roberto
Miniero
M
D006402:Hematologic Diseases / Q000209:etiology*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009196:Myeloproliferative Disorders / Q000209:etiology*; D009634:Noonan Syndrome / Q000175:diagnosis*