[Systemic mastocytosis--definition of an internal disease]

Med Klin (Munich). 2010 Aug;105(8):544-53. doi: 10.1007/s00063-010-1093-8. Epub 2010 Sep 8.
[Article in German]

Abstract

Systemic mastocytosis comprises disorders characterized by an accumulation of genetically altered mast cells in all organs and tissues due to an increased proliferation rate and reduced apoptosis of those pathologic mast cells. Release of their mediators can effectively influence organ function and can lead to systemic effects without inducing traces in routinely used laboratory parameters or imaging methods. In most cases, little invasive investigations allow diagnosing the disease and, hence, an appropriate therapy consisting of a basic medication with antihistamine and mast cell membrane-stabilizing compounds that should be supplemented, if required, by a medication adapted to individual symptoms, can be initiated. Because of the probably high prevalence of the disorder, systemic mastocytosis should be considered as a differential diagnosis in particular in the case of chronic gastrointestinal complaints such as abdominal pain/discomfort possibly associated with diarrhea, at an early stage.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Algorithms
  • Apoptosis / physiology
  • Ascorbic Acid / administration & dosage
  • Biopsy
  • Bone Marrow / pathology
  • Codon / genetics
  • Combined Modality Therapy
  • Cromolyn Sodium / therapeutic use
  • DNA Mutational Analysis
  • Delayed-Action Preparations
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Gastrointestinal Diseases / physiopathology
  • Histamine H1 Antagonists / therapeutic use
  • Histamine H2 Antagonists / therapeutic use
  • Humans
  • Ketotifen / therapeutic use
  • Mast Cells / physiology
  • Mastocytosis / diagnosis*
  • Mastocytosis / drug therapy
  • Mastocytosis / genetics
  • Mastocytosis / physiopathology
  • Phenotype
  • Proto-Oncogene Proteins c-kit / genetics

Substances

  • Codon
  • Delayed-Action Preparations
  • Histamine H1 Antagonists
  • Histamine H2 Antagonists
  • Proto-Oncogene Proteins c-kit
  • Ascorbic Acid
  • Cromolyn Sodium
  • Ketotifen