Systemic mastocytosis.

Hans-Peter Horny, Tracy I George

Journal: Hematology/oncology clinics of North America 2012;25(5):1067-83, vii

PMID: 22054735

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Abstract

An unusual disease, mastocytosis challenges the pathologist with a variety of morphologic appearances and heterogeneous clinical presentations ranging from skin manifestations (pruritus, urticaria, dermatographism) to systemic signs and symptoms indicative of mast cell mediator release, including flushing, hypotension, headache, and anaphylaxis among others. In this article, we focus on recognizing the cytology, histopathology, clinical features, and prognostic implications of systemic mastocytosis, a clonal and neoplastic mast cell proliferation infiltrating extracutaneous organ(s) with or without skin involvement. Diagnostic pitfalls are reviewed with ancillary studies to help unmask the mast cell and exclude morphologic mimics.

Address: Department of Pathology, Stanford University School of Medicine, Stanford University Medical Center, 300 Pasteur Drive, Room H1501B, Stanford, CA 94305-5627, USA. [email protected]

DOI: 10.1016/j.hoc.2011.09.012

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MeSH Terms: Amino Acid Substitution, Humans, Mast Cells, Mastocytosis, Systemic, Mutation, Missense, Myeloproliferative Disorders, Prognosis, Proto-Oncogene Proteins c-kit, World Health Organization