Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: response to corticotherapy.
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.
Michel
G
G
Pediatric Hematology Department, Children's Hospital La Timone, Marseille, France.
Thuret
I
I
Capodano
A M
AM
Scheiner
C
C
Guitard
A M
AM
Mozziconacci
M J
MJ
Fossat
C
C
Perrimond
H
H
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
D011241:Prednisone