Pediatric 8p11 eosinophilic myeloproliferative syndrome (EMS): A case report and review of the literature.

Document Type

Article

Publication Date

5-1-2017

Identifier

DOI: 10.1002/pbc.26310

Abstract

The 8p11 eosinophilic myeloproliferative syndrome (EMS) is an aggressive neoplasm driven by translocation of the fibroblast growth factor receptor 1 and often transforms to leukemias and lymphomas that are refractory to treatment. The first case was identified in 1983, and to date over 70 cases have been reported in the literature. Despite those reports, no consensus exists on management of this condition, and inconsistency in treatment regimens is even more pronounced in the pediatric literature. We report a case of a male infant with the 8p11 EMS, review the published pediatric experience with EMS, and discuss treatment strategies for this enigmatic hematological disorder.

Journal Title

Pediatric blood & cancer

Volume

64

Issue

5

MeSH Keywords

Adolescent; Adult; Antiviral Agents; Bone Marrow Transplantation; Child; Child, Preschool; Chromosomes, Human, Pair 8; Eosinophilia; Female; Ganciclovir; Herpesvirus 6, Human; Humans; Infant; Male; Myeloproliferative Disorders; Receptor, Fibroblast Growth Factor, Type 1; Roseolovirus Infections; Young Adult

Keywords

FGFR translocation; eosinophilia; myeloproliferative neoplasm; cancer; Fibroblast Growth Factor Receptor

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