title: A case of malignant mastocytosis with circulating mast cell precursors: biologic and phenotypic characterization of the malignant clone.
abstract: The phenotypic and biologic properties of malignant cells in a case of aggressive mastocytosis with multi-organ involvement, circulating mast cell precursors and absence of skin infiltrates were analyzed. Circulating mast cell precursors were detected by immunostaining using antibodies against mast cell tryptase as well as by electron microscopy. These progenitors were tryptase+/chymase- (MCT) and accounted for 10 to 20% of nucleated mononuclear blood cells (MNC). A subset of them contained metachromatic granules. As assessed by combined toluidine blue/immunofluorescence staining, the granulated mast cell precursors were found to express CD9 (P24), CD33 (gp67) and CD44 (Pgp-1), but not basophil-related markers (CD11b (C3biR), CDw17 (lactosylceramide), CD123 (il-3R alpha))or monocyte-related antigens (CD14, CD15). Expression of the mast cell growth factor (MGF) receptor, c-kit(CD117), was also demonstrable, whereas the skin mast cell marker C5aR (CD88) could not be detected on mast cell precursors. The ligand of c-kit, recombinant human (rh) stem cell factor (SCF = MGF), induced histamine release from circulating mast cell progenitors, whereas rhC5a, a potent skin mast cell-/basophil-agonist, was ineffective over the dose-range (10(-9) to 10(-7(M)) tested. Analysis of mast cell antigens in malignant mastocytosis or mast cell leukemias may be helpful to establish a diagnosis and to determine the phenotype of the clone.
authors.affiliation: Department of Internal Medicine I, University of Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Baghestanian
authors.forename: H c
authors.initials: Hc
authors.lastname: Bankl
authors.forename: C
authors.initials: C
authors.lastname: Sillaber
authors.forename: W J
authors.initials: WJ
authors.lastname: Beil
authors.forename: T
authors.initials: T
authors.lastname: Radaszkiewicz
authors.forename: W
authors.initials: W
authors.lastname: Füreder
authors.forename: J
authors.initials: J
authors.lastname: Preiser
authors.forename: M
authors.initials: M
authors.lastname: Vesely
authors.forename: G
authors.initials: G
authors.lastname: Schernthaner
authors.forename: K
authors.initials: K
authors.lastname: Lechner
authors.forename: P
authors.initials: P
authors.lastname: Valent
mesh_terms: D000328:Adult; D053818:Chymases; D003594:Cytoplasmic Granules / Q000473:pathology; D006412:Hematopoietic Stem Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D006636:Histamine Release; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008297:Male; D008407:Mast Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D012515:Mast-Cell Sarcoma / Q000097:blood / Q000276:immunology / Q000473:pathology*; D008854:Microscopy, Electron; D014411:Neoplastic Stem Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D012697:Serine Endopeptidases / Q000378:metabolism; D019089:Stem Cell Factor / Q000378:metabolism / Q000494:pharmacology; D053802:Tryptases
chemical_list: D019089:Stem Cell Factor; D019009:Proto-Oncogene Proteins c-kit; D012697:Serine Endopeptidases; C083294:chymase 2; D053818:Chymases; D053802:Tryptases