Partial Response to Sorafenib in a Child With a Myeloid/Lymphoid Neoplasm, Eosinophilia, and a ZMYM2-FLT3 Fusion

J Pediatr Hematol Oncol. 2021 May 1;43(4):e508-e511. doi: 10.1097/MPH.0000000000001890.

Abstract

Dysregulated tyrosine kinases in myeloid/lymphoid neoplasms with eosinophilia are rare, but do occur in children. To increase awareness of this diagnosis, we present a child who was diagnosed after a 3-year disease history. The patient was initially treated according to a T-cell lymphoblastic lymphoma protocol, but genetic analyses at recurrence revealed microdeletions resulting in an in-frame fusion of ZMYM2 and FLT3. Treatment with sorafenib, an FLT3 tyrosine kinase inhibitor, rapidly resulted in significant reduction of lymphadenopathy and normalization of white blood cell and eosinophil counts. At 17 months of treatment, he remains in complete hematologic, but not molecular remission.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Agents / therapeutic use*
  • Child, Preschool
  • Eosinophilia / complications
  • Humans
  • Lymphoma / complications
  • Lymphoma / drug therapy*
  • Lymphoma / genetics
  • Male
  • Nuclear Proteins / genetics*
  • Oncogene Proteins, Fusion / genetics
  • Protein Kinase Inhibitors / therapeutic use
  • Sorafenib / therapeutic use*
  • fms-Like Tyrosine Kinase 3 / genetics*

Substances

  • Antineoplastic Agents
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • Protein Kinase Inhibitors
  • ZMYM3 protein, human
  • Sorafenib
  • FLT3 protein, human
  • fms-Like Tyrosine Kinase 3