Slowly progressive systemic mastocytosis with high mast-cell burden and no evidence of a non-mast-cell hematologic disorder: an example of a smoldering case?

Leuk Res. 2001 Jul;25(7):635-8. doi: 10.1016/s0145-2126(01)00023-6.

Abstract

A 43-year-old man with extensive systemic mastocytosis with poor prognostic indicators but no overt hematologic abnormality is described. This patient's clinical presentation and course are consistent with the newly proposed 'smoldering mastocytosis' category. Long-term follow-up of patients is needed to determine whether they may be at higher risk for progression into more aggressive categories.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amino Acid Substitution
  • Biomarkers, Tumor / analysis
  • Bone Diseases, Metabolic / etiology
  • Bone Marrow / pathology
  • CD2 Antigens / analysis
  • Cell Count
  • Cell Transformation, Neoplastic / pathology
  • DNA Mutational Analysis
  • Disease Progression
  • Hepatomegaly / etiology
  • Humans
  • Male
  • Mast Cells / chemistry
  • Mast Cells / pathology
  • Mastocytosis / blood
  • Mastocytosis / complications
  • Mastocytosis / genetics
  • Mastocytosis / pathology*
  • Mutation, Missense
  • Pancytopenia / etiology
  • Proto-Oncogene Proteins c-kit / genetics
  • Receptors, Complement 3b / analysis
  • Receptors, Interleukin-2 / analysis
  • Splenomegaly / etiology
  • Urticaria Pigmentosa / blood
  • Urticaria Pigmentosa / pathology

Substances

  • Biomarkers, Tumor
  • CD2 Antigens
  • Receptors, Complement 3b
  • Receptors, Interleukin-2
  • Proto-Oncogene Proteins c-kit