Journal
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
Volume 25, Issue 5, Pages 1067-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.hoc.2011.09.012
Keywords
Systemic mastocytosis; Mast cell; KIT D816V; Mast cell leukemia
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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.
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