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

Pediatr Blood Cancer. 2017 May;64(5). doi: 10.1002/pbc.26310. Epub 2016 Nov 3.

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.

Keywords: FGFR translocation; eosinophilia; myeloproliferative neoplasm.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Antiviral Agents / therapeutic use
  • Bone Marrow Transplantation*
  • Child
  • Child, Preschool
  • Chromosomes, Human, Pair 8 / genetics*
  • Eosinophilia / genetics*
  • Eosinophilia / therapy*
  • Female
  • Ganciclovir / therapeutic use
  • Herpesvirus 6, Human / isolation & purification
  • Humans
  • Infant
  • Male
  • Myeloproliferative Disorders / genetics*
  • Myeloproliferative Disorders / therapy*
  • Receptor, Fibroblast Growth Factor, Type 1 / genetics*
  • Roseolovirus Infections / diagnosis
  • Roseolovirus Infections / drug therapy
  • Young Adult

Substances

  • Antiviral Agents
  • FGFR1 protein, human
  • Receptor, Fibroblast Growth Factor, Type 1
  • Ganciclovir

Supplementary concepts

  • Myeloproliferative Disorder, Chronic, with Eosinophilia