Mastocytosis

S. Fiona Bonar, Edward F McCarthy

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Mastocytosis is a generic term referring to a spectrum of rare disorders characterized by the proliferation of mast cells in tissues. Mastocytosis in the bone is a very rare, systemic disease usually limited to adults. Osseous changes are the most constant abnormality in systemic mastocytosis. Lesions involve both the axial and appendicular skeleton and may be diffuse or focal. Rarely, patients with osseous disease lack cutaneous involvement. Patients may present weight loss, weakness, and hematologic abnormalities due to replacement of the bone marrow. Bone lesions are usually asymptomatic but may present bone pain. Both radiolytic and radiodense lesions occur. Radiolytic changes may be focal or diffuse. Histologically, there is focal or diffuse infi ltration by mast cells, admixed with other marrow elements, including eosinophils and fi broblasts, or in solid sheets. Immunostain for mast cell tryptase is positive. Patients with “bone-only” disease follow an indolent course. Treatment is supportive and is geared to reducing systemic symptoms.

Original languageEnglish (US)
Title of host publicationTumors and Tumor-Like Lesions of Bone: For Surgical Pathologists, Orthopedic Surgeons and Radiologists
PublisherSpringer-Verlag London Ltd
Pages939-979
Number of pages41
ISBN (Print)9781447165781, 9781447165774
DOIs
StatePublished - Jan 1 2015

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

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