Pediatric 8p11 eosinophilic myeloproliferative syndrome (EMS): A case report and review of the literature.
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.
Children's Mercy Hospitals and Clinics, Kansas City, Missouri 64113.
Jay F
JF
Sarthy
Children's Mercy Hospitals and Clinics, Kansas City, Missouri 64113.
Naresh
N
Reddivalla
Children's Mercy Hospitals and Clinics, Kansas City, Missouri 64113.
Mohamed
M
Radhi
Children's Mercy Hospitals and Clinics, Kansas City, Missouri 64113.
Katherine
K
Chastain
D000293:Adolescent; D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics*; D004802:Eosinophilia / Q000235:genetics* / Q000628:therapy*; D005260:Female; D015774:Ganciclovir / Q000627:therapeutic use; D015654:Herpesvirus 6, Human / Q000302:isolation & purification; D006801:Humans; D007223:Infant; D008297:Male; D009196:Myeloproliferative Disorders / Q000235:genetics* / Q000628:therapy*; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics*; D019349:Roseolovirus Infections / Q000175:diagnosis / Q000188:drug therapy; D055815:Young Adult
D000998:Antiviral Agents; C496348:FGFR1 protein, human; D051496:Receptor, Fibroblast Growth Factor, Type 1; D015774:Ganciclovir
FGFR translocation; eosinophilia; myeloproliferative neoplasm