Systemic mastocytosis with associated myeloproliferative neoplasm with t(8;19)(p12;q13.1) and abnormality of FGFR1: report of a unique case

Int J Clin Exp Pathol. 2014 Jan 15;7(2):801-7. eCollection 2014.

Abstract

Systemic mastocytosis is a neoplastic proliferation of mast cells that frequently presents with associated clonal hematological non-mast cell lineage disease. Myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene are a heterogenous group of rare and aggressive hematopoietic stem cell disorders. About a dozen of chromosome changes involving the FGFR1 gene, presenting as myeloid or lymphoid neoplasms, have been described in the literature. To date, only 2 cases of myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene have been reported in association with systemic mastocytosis, one with t(8;13) and one with t(8;17) involving the FGFR1 gene. Here we describe another case of myeloproliferative neoplasm with chromosome translocation t(8;19) involving FGFR1 gene associated with systemic mastocytosis.

Keywords: FGFR1; Myeloproliferative neoplasm; eosinophilia; systemic mastocytosis associated with clonal hematological non-mast cell lineage disease.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics*
  • Biopsy
  • Bone Marrow Examination
  • Chromosomes, Human, Pair 19*
  • Chromosomes, Human, Pair 8*
  • Gene Rearrangement*
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Karyotyping
  • Male
  • Mastocytosis, Systemic / genetics*
  • Mastocytosis, Systemic / metabolism
  • Mastocytosis, Systemic / pathology
  • Mastocytosis, Systemic / surgery
  • Myeloproliferative Disorders / genetics*
  • Myeloproliferative Disorders / metabolism
  • Myeloproliferative Disorders / pathology
  • Myeloproliferative Disorders / surgery
  • Phenotype
  • Receptor, Fibroblast Growth Factor, Type 1 / genetics*
  • Stem Cell Transplantation
  • Translocation, Genetic*
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • FGFR1 protein, human
  • Receptor, Fibroblast Growth Factor, Type 1