Aggressive systemic mastocytosis

Joint Bone Spine. 2003 Feb;70(1):64-6. doi: 10.1016/s1297-319x(02)00015-5.

Abstract

Systemic mastocytosis is a rare and occasionally aggressive condition that raises major diagnostic challenges. We report a case in a 72-year-old patient in whom the diagnosis of malignant mastocytosis required two bone marrow smears and three bone marrow biopsies examined using specific staining techniques. Despite interferon therapy, a mast-cell sarcoma of the sternum developed 1 year after symptom onset, followed 1 year later by acute myeloblastic leukemia, which was rapidly fatal.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Alendronate / therapeutic use
  • Bone Marrow Cells / enzymology
  • Bone Marrow Cells / pathology
  • Fatal Outcome
  • Female
  • Humans
  • Interferon-alpha / therapeutic use
  • Leukemia, Myeloid, Acute / pathology
  • Mast Cells / enzymology
  • Mast Cells / pathology
  • Mast-Cell Sarcoma / pathology
  • Mastocytosis, Systemic / complications
  • Mastocytosis, Systemic / pathology*
  • Mastocytosis, Systemic / therapy
  • Neoplasms, Multiple Primary
  • Osteoporosis, Postmenopausal / drug therapy
  • Prednisone / therapeutic use
  • Serine Endopeptidases / metabolism
  • Tryptases

Substances

  • Interferon-alpha
  • Serine Endopeptidases
  • Tryptases
  • Prednisone
  • Alendronate