title: Mastocytosis: immunophenotypical features of the transformed mast cells are unique among hematopoietic cells.
abstract: Mastocytosis is a disease of bone marrow origin histologically characterized by compact tissue infiltrates of atypical mast cells never seen in reactive states. Most patients with mastocytosis have transformed mast cells carrying an activating point mutation at codon 816 of KIT and also show an elevated serum tryptase level. In this article immunophenotypical features of mast cells are described. Based on these features, mast cells are not closely related to other myeloid cells. Using the knowledge on aberrantly expressed antigens by mast cells, the hematopathologist should be able to recognize the disease even in the presence of unusual morphologic findings or an associated hematologic non-mast cell lineage disease.
authors.affiliation: Institute of Pathology, Ludwig-Maximilians-Universität, Thalkirchnerstrasse 36, D-80337 Munich, Germany. Electronic address: Hans-Peter.Horny@med.uni-muenchen.de.
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Horny
authors.affiliation: Institute of Pathology, Ludwig-Maximilians-Universität, Thalkirchnerstrasse 36, D-80337 Munich, Germany.
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine, 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; D015703:Antigens, CD / Q000235:genetics / Q000276:immunology*; D001853:Bone Marrow / Q000276:immunology* / Q000473:pathology; D002648:Child; D015870:Gene Expression; D006801:Humans; D016130:Immunophenotyping; D008407:Mast Cells / Q000276:immunology* / Q000473:pathology; D008415:Mastocytosis / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D009154:Mutation; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000276:immunology; D053802:Tryptases / Q000235:genetics / Q000276:immunology
chemical_list: D015703:Antigens, CD; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases
keywords: CD117; CD30; Immunophenotype; Mast cell; Mast cell leukemia; Mastocytosis; Systemic mastocytosis; Tryptase
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