title: Diagnostic criteria and classification of mastocytosis in 2014.
abstract: Mastocytosis is characterized by accumulation of pathologic mast cells in tissues. Most patients with mastocytosis experience mast cell activation symptoms in response to various triggers. The diagnosis of mastocytosis should be made from objective pathologic findings. Modern diagnostic criteria and classification of mastocytosis were proposed in 2000 by an international consensus group and formed the basis of the current World Health Organization (WHO) guidelines, which have been validated to correlate with prognosis and help selection of therapy. In this article, the WHO criteria for diagnosis and classification are summarized and practical aspects to avoid common pitfalls in diagnostic workup are discussed.
authors.affiliation: Harvard Medical School, Director, Mastocytosis Center, Brigham and Women's Hospital, Harvard Medical School, 1 Jimmy Fund Way, Room 626B, Boston, MA 02115, USA. Electronic address: cakin@partners.org.
authors.forename: Cem
authors.initials: C
authors.lastname: Akin
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Währinger Gürtel 18-20, A-1090, Vienna, Austria.
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
mesh_terms: D000328:Adult; D000367:Age Factors; D015415:Biomarkers / Q000378:metabolism; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology*; D002648:Child; D041981:Gastrointestinal Tract / Q000276:immunology / Q000473:pathology; D015870:Gene Expression; D006801:Humans; D053645:Interleukin-2 Receptor alpha Subunit / Q000235:genetics / Q000276:immunology; D008407:Mast Cells / Q000276:immunology / Q000473:pathology*; D008415:Mastocytosis / Q000145:classification / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D009154:Mutation; D017410:Practice Guidelines as Topic; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000276:immunology; D012867:Skin / Q000276:immunology / Q000473:pathology*; D053802:Tryptases / Q000097:blood / Q000235:genetics
chemical_list: D015415:Biomarkers; C508017:IL2RA protein, human; D053645:Interleukin-2 Receptor alpha Subunit; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases
keywords: Classification; Diagnosis; Mastocytosis; Tryptase; World Health Organization; c-kit