Response of ETV6-FLT3-positive myeloid/lymphoid neoplasm with eosinophilia to inhibitors of FMS-like tyrosine kinase 3

Blood. 2011 Aug 25;118(8):2239-42. doi: 10.1182/blood-2011-03-343426. Epub 2011 Jun 24.

Abstract

Imatinib-resistant tyrosine kinase (TK) fusions involving FGFR1, JAK2, or FLT3 are rare but recurrent in patients with eosinophilia-associated neoplasms. We report here 2 male patients with ETV6-FLT3(+) myeloid/lymphoid neoplasms with eosinophilia who were treated with the multitargeted TK inhibitors sunitinib and sorafenib. Patient 1 achieved rapid complete hematologic response and complete cytogenetic response after 3 months of taking sunitinib. A secondary blast phase caused by clonal evolution was diagnosed after 6 months. He achieved a second complete hematologic response after taking sorafenib but relapsed 2 months later. An N841K point mutation within the TK domain of FLT3, previously reported in acute myeloid leukemia and potentially conferring resistance to sorafenib, was subsequently identified. Patient 2 was heavily pretreated according to the initial diagnosis of T-lymphoblastic lymphoma and died in sunitinib-induced pancytopenia. This report highlights the importance of a careful diagnostic workup for eosinophilia-associated neoplasms to evaluate the possibility of TK inhibitor therapy.

Publication types

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

MeSH terms

  • Adult
  • Amino Acid Sequence
  • Antineoplastic Agents / therapeutic use
  • Base Sequence
  • Benzenesulfonates / therapeutic use
  • DNA, Neoplasm / genetics
  • Drug Resistance, Neoplasm / genetics
  • ETS Translocation Variant 6 Protein
  • Eosinophilia / complications
  • Eosinophilia / drug therapy
  • Eosinophilia / genetics
  • Hematologic Neoplasms / complications
  • Hematologic Neoplasms / drug therapy*
  • Hematologic Neoplasms / genetics*
  • Humans
  • Indoles / therapeutic use
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Niacinamide / analogs & derivatives
  • Oncogene Fusion
  • Oncogene Proteins, Fusion / genetics*
  • Phenylurea Compounds
  • Point Mutation
  • Protein Kinase Inhibitors / therapeutic use
  • Proto-Oncogene Proteins c-ets / genetics*
  • Pyridines / therapeutic use
  • Pyrroles / therapeutic use
  • Repressor Proteins / genetics*
  • Sorafenib
  • Sunitinib
  • fms-Like Tyrosine Kinase 3 / antagonists & inhibitors*
  • fms-Like Tyrosine Kinase 3 / genetics*

Substances

  • Antineoplastic Agents
  • Benzenesulfonates
  • DNA, Neoplasm
  • Indoles
  • Oncogene Proteins, Fusion
  • Phenylurea Compounds
  • Protein Kinase Inhibitors
  • Proto-Oncogene Proteins c-ets
  • Pyridines
  • Pyrroles
  • Repressor Proteins
  • Niacinamide
  • Sorafenib
  • FLT3 protein, human
  • fms-Like Tyrosine Kinase 3
  • Sunitinib