Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: Response to corticotherapy
✍ Scribed by Michel, G. ;Thuret, I. ;Capodano, A. M. ;Scheiner, C. ;Guitard, A. M. ;Mozziconacci, M. J. ;Fossat, C. ;Perrimond, H.
- Publisher
- John Wiley and Sons
- Year
- 1991
- Tongue
- English
- Weight
- 447 KB
- Volume
- 19
- Category
- Article
- ISSN
- 0098-1532
No coin nor oath required. For personal study only.
✦ Synopsis
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.