title: Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: response to corticotherapy.
abstract: The idiopathic hypereosinophilic syndrome (IHS) is extremely rare in childhood and relationships of this syndrome with myeloproliferative diseases are controversial. We reported the observation of a 7-year-old girl suffering from an IHS with myelofibrosis. A clonal cytogenetic abnormality, trisomy 8, was detected in the bone marrow cells of this child. This is the decisive proof of a myeloproliferative disorder. IHS with myelofibrosis is usually considered as unresponsive to corticotherapy. In our case, corticotherapy resulted in a rapid, complete, and lasting disappearance of myelofibrosis. Complete remission of the disease, however, was not achieved and the trisomy 8 persisted after treatment.
authors.affiliation: Pediatric Hematology Department, Children's Hospital La Timone, Marseille, France.
authors.forename: G
authors.initials: G
authors.lastname: Michel
authors.forename: I
authors.initials: I
authors.lastname: Thuret
authors.forename: A M
authors.initials: AM
authors.lastname: Capodano
authors.forename: C
authors.initials: C
authors.lastname: Scheiner
authors.forename: A M
authors.initials: AM
authors.lastname: Guitard
authors.forename: M J
authors.initials: MJ
authors.lastname: Mozziconacci
authors.forename: C
authors.initials: C
authors.lastname: Fossat
authors.forename: H
authors.initials: H
authors.lastname: Perrimond
mesh_terms: D002648:Child; D002898:Chromosomes, Human, Pair 8; D004802:Eosinophilia / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D005260:Female; D006801:Humans; D007958:Leukocyte Count / Q000187:drug effects; D011241:Prednisone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D013577:Syndrome; D014314:Trisomy
chemical_list: D011241:Prednisone