title: Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment.
abstract: Recent advances in our understanding of mast cell biology and disease resulted in identification of important differences in expression of mast cell surface antigens in normal and neoplastic mast cells. Most notably, detection of aberrant expression of CD25 and CD2 on the surface of neoplastic mast cells but not on their normal counterparts leads to the inclusion of this immunophenotypic abnormality in the World Health Organization's diagnostic criteria for systemic mastocytosis. Aberrant mast cell surface marker expression can be detected in the bone marrow aspirate by flow cytometry, even in patients with limited disease that lacks histopathologically detectable aggregates of mast cells in bone marrow biopsy sections. Flow cytometric analysis of bone marrow mast cells is therefore a sensitive method of diagnosis of mast cell disease and is expected to find increasing use in determining response to emerging mast cell cytoreductive therapies.
authors.affiliation: Department of Internal Medicine, University of Michigan, 4220-D MSRB-3, Box 0638, 1150 West Medical Center Drive, Ann Arbor, MI 48109-0638, USA. cemakin@umich.edu
authors.forename: Cem
authors.initials: C
authors.lastname: Akin
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
authors.forename: Luis
authors.initials: L
authors.lastname: Escribano
mesh_terms: D014408:Biomarkers, Tumor / Q000096:biosynthesis; D001706:Biopsy; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D018801:CD2 Antigens / Q000096:biosynthesis; D003937:Diagnosis, Differential; D015972:Gene Expression Regulation, Neoplastic; D016130:Immunophenotyping / Q000379:methods; D008407:Mast Cells / Q000378:metabolism / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D051918:Sialic Acid Binding Ig-like Lectin 2 / Q000096:biosynthesis; D014582:Urticaria Pigmentosa / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000628:therapy*
chemical_list: D014408:Biomarkers, Tumor; D018801:CD2 Antigens; D051918:Sialic Acid Binding Ig-like Lectin 2