mastocytosis

Summary

Summary: A heterogenous group of disorders characterized by the abnormal increase of MAST CELLS in only the skin (MASTOCYTOSIS, CUTANEOUS), in extracutaneous tissues involving multiple organs (MASTOCYTOSIS, SYSTEMIC), or in solid tumors (MASTOCYTOMAS).

Top Publications

  1. ncbi Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-x(L)
    J H Jordan
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Vienna, Austria
    Hum Pathol 32:545-52. 2001
  2. ncbi The mast cell in interstitial cystitis: role in pathophysiology and pathogenesis
    Grannum R Sant
    Department of Urology, Tufts University School of Medicine, Boston, Massachusetts, USA
    Urology 69:34-40. 2007
  3. ncbi The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations
    Yongsheng Ma
    Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Blood 99:1741-4. 2002
  4. ncbi KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients
    Andres C Garcia-Montero
    Centro de Investigacion del Cancer, Campus Miguel de Unamuno, 37007 Salamanca, Spain
    Blood 108:2366-72. 2006
  5. ncbi Recent advances in the understanding of mastocytosis: the role of KIT mutations
    Alberto Orfao
    Centro de Investigación del Cáncer IBMCC, Departamento de Medicina and Servicio General de Citometría, University of Salamanca, Salamanca, Spain
    Br J Haematol 138:12-30. 2007
  6. ncbi IL-9-deficient mice establish fundamental roles for IL-9 in pulmonary mastocytosis and goblet cell hyperplasia but not T cell development
    J M Townsend
    MRC Laboratory of Molecular Biology, Cambridge, United Kingdom
    Immunity 13:573-83. 2000
  7. ncbi In aggressive forms of mastocytosis, TET2 loss cooperates with c-KITD816V to transform mast cells
    Erinn Soucie
    Inserm U1068, Centre de Recherche en Cancerologie de Marseille, centre de référence des mastocytoses, Institut Paoli Calmettes, Universite de la Mediterranee, Marseille, France
    Blood 120:4846-9. 2012
  8. pmc Apoptosis, mastocytosis, and diminished adipocytokine gene expression accompany reduced epididymal fat mass in long-standing diet-induced obese mice
    Mehmet M Altintas
    Department of Medicine, Miller School of Medicine, University of Miami, Miami, FL, USA
    Lipids Health Dis 10:198. 2011
  9. pmc Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder
    H Nagata
    Allergic Diseases Section, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 92:10560-4. 1995
  10. ncbi Diagnostic criteria and classification of mastocytosis: a consensus proposal
    P Valent
    Department of Internal Medicine I, Division of Hematology, University of Vienna, Währinger Gürtel 18 20 Vienna, Austria
    Leuk Res 25:603-25. 2001

Detail Information

Publications236 found, 100 shown here

  1. ncbi Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-x(L)
    J H Jordan
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Vienna, Austria
    Hum Pathol 32:545-52. 2001
    ..markers for mast cell (MC)-proliferative disorders, serial bone marrow (bm) sections of 22 patients with mastocytosis (systemic indolent mastocytosis, n = 19; mast cell leukemia [MCL], n = 1; isolated bm mastocytosis, n = 2) were ..
  2. ncbi The mast cell in interstitial cystitis: role in pathophysiology and pathogenesis
    Grannum R Sant
    Department of Urology, Tufts University School of Medicine, Boston, Massachusetts, USA
    Urology 69:34-40. 2007
    ....
  3. ncbi The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations
    Yongsheng Ma
    Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Blood 99:1741-4. 2002
    ..of c-KIT causing spontaneous activation of the KIT receptor kinase are associated with sporadic adult human mastocytosis (SAHM) and with human gastrointestinal stromal tumors...
  4. ncbi KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients
    Andres C Garcia-Montero
    Centro de Investigacion del Cancer, Campus Miguel de Unamuno, 37007 Salamanca, Spain
    Blood 108:2366-72. 2006
    ..of KIT mutations in different compartments of bone marrow (BM) hematopoietic cells of individuals with systemic mastocytosis (SM), and its different diagnostic categories, remains unknown...
  5. ncbi Recent advances in the understanding of mastocytosis: the role of KIT mutations
    Alberto Orfao
    Centro de Investigación del Cáncer IBMCC, Departamento de Medicina and Servicio General de Citometría, University of Salamanca, Salamanca, Spain
    Br J Haematol 138:12-30. 2007
    b>Mastocytosis is a heterogeneous disorder characterised by the expansion and accumulation of mast cells in different organs and tissues...
  6. ncbi IL-9-deficient mice establish fundamental roles for IL-9 in pulmonary mastocytosis and goblet cell hyperplasia but not T cell development
    J M Townsend
    MRC Laboratory of Molecular Biology, Cambridge, United Kingdom
    Immunity 13:573-83. 2000
    ..a distinct requirement for IL-9 in the rapid and robust generation of pulmonary goblet cell hyperplasia and mastocytosis in response to lung challenge. In contrast, eosinophilia and granuloma formation were not affected...
  7. ncbi In aggressive forms of mastocytosis, TET2 loss cooperates with c-KITD816V to transform mast cells
    Erinn Soucie
    Inserm U1068, Centre de Recherche en Cancerologie de Marseille, centre de référence des mastocytoses, Institut Paoli Calmettes, Universite de la Mediterranee, Marseille, France
    Blood 120:4846-9. 2012
    ..We now show, using a large cohort of mastocytosis patients, including an almost equal number of aggressive and nonaggressive cases of systemic mastocytosis, that ..
  8. pmc Apoptosis, mastocytosis, and diminished adipocytokine gene expression accompany reduced epididymal fat mass in long-standing diet-induced obese mice
    Mehmet M Altintas
    Department of Medicine, Miller School of Medicine, University of Miami, Miami, FL, USA
    Lipids Health Dis 10:198. 2011
    ..Moreover, gene and/or protein expression of several adipocytokines (leptin, adiponectin, TNF-α, IL-10, IL-6, MCP-1), F4/80, mMCP6, cleaved caspase-3 were determined...
  9. pmc Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder
    H Nagata
    Allergic Diseases Section, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 92:10560-4. 1995
    ..These observations suggested to us that mastocytosis might in some patients result from mutations in c-kit...
  10. ncbi Diagnostic criteria and classification of mastocytosis: a consensus proposal
    P Valent
    Department of Internal Medicine I, Division of Hematology, University of Vienna, Währinger Gürtel 18 20 Vienna, Austria
    Leuk Res 25:603-25. 2001
    The term 'mastocytosis' denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in one or more organ systems...
  11. ncbi Mastocytosis: state of the art
    Hans Peter Horny
    Institute of Pathology, Ansbach, Germany
    Pathobiology 74:121-32. 2007
    b>Mastocytosis is a neoplastic disease involving mast cells (MC) and their CD34+ progenitors...
  12. pmc Mast cells are required for experimental oral allergen-induced diarrhea
    Eric B Brandt
    Division of Allergy and Immunology, Department of Pediatrics, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio 45229, USA
    J Clin Invest 112:1666-77. 2003
    ..restricted, dose-dependent, acute diarrhea associated with increased intestinal permeability, eosinophilia, and mastocytosis. Mice developed limited systemic manifestations of anaphylaxis, even though they developed marked intestinal ..
  13. pmc Duodenal mastocytosis, eosinophilia and intraepithelial lymphocytosis as possible disease markers in the irritable bowel syndrome and functional dyspepsia
    M M Walker
    Department of Histopathology, Faculty of Medicine, St Mary s Campus, Imperial College, London, UK
    Aliment Pharmacol Ther 29:765-73. 2009
    ..Irritable bowel syndrome (IBS) and functional dyspepsia (FD) are common functional disorders without defined pathology. Mast cells and eosinophils interact with T lymphocytes and may alter enteric nerve and smooth muscle function...
  14. ncbi Mastocytosis
    Luciana Cirillo Maluf
    Mestre em Ciências da Saúde pela Faculdade de Medicina do ABC, Preceptor da disciplina de Dermatologia da Faculdade de Medicina do ABC, Santo Andre SP, Brazil
    An Bras Dermatol 84:213-25. 2009
    b>Mastocytosis is characterized by pathologic accumulation and activation of mast cells in tissues and organs...
  15. ncbi Diagnosis of mastocytosis: value of cytochemistry and immunohistochemistry
    C Y Li
    Hematopathology Hilton 1020, Mayo Clinic, 200 First Street, Southwest, Rochester, MN 55905, USA
    Leuk Res 25:537-41. 2001
    The diagnosis of mastocytosis or mast cell disease may be difficult sometimes because of the wide variety of clinical presentation, abnormal morphology of mast cells, and variation in histologic features which may mimic varieties of other ..
  16. ncbi A novel K509I mutation of KIT identified in familial mastocytosis-in vitro and in vivo responsiveness to imatinib therapy
    Ling Yan Zhang
    Wessex Regional Genetics Laboratory, Salisbury, UK
    Leuk Res 30:373-8. 2006
    KIT mutation has been implicated in sporadic mastocytosis, yet clusters in only a few sites in the molecule...
  17. ncbi Immunophenotypic analysis of mast cells in mastocytosis: When and how to do it. Proposals of the Spanish Network on Mastocytosis (REMA)
    Luis Escribano
    Unidad de Mastocitosis, Servicio de Hematología and Laboratorio K Frank Austen, Hospital Ramon y Cajal, Madrid, Spain
    Cytometry B Clin Cytom 58:1-8. 2004
    b>Mastocytosis is a term used for a heterogeneous group of disorders characterized by an abnormal proliferation and accumulation of mast cells (MCs) in one or multiple tissues including skin, bone marrow, liver, spleen, and lymph nodes, ..
  18. ncbi Evaluation of the WHO criteria for the classification of patients with mastocytosis
    Laura Sánchez-Muñoz
    Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain
    Mod Pathol 24:1157-68. 2011
    Diagnosis and classification of mastocytosis is currently based on the World Health Organization (WHO) criteria...
  19. ncbi Immunophenotypic characterization of bone marrow mast cells in mastocytosis and other mast cell disorders
    Laura Sánchez-Muñoz
    Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain
    Methods Cell Biol 103:333-59. 2011
    b>Mastocytosis is a term used to designate a heterogeneous group of disorders characterized by an abnormal proliferation and accumulation of mast cells (MCs) in one or multiple tissues including skin, bone marrow (BM), liver, spleen, and ..
  20. ncbi Somatic c-KIT activating mutation in urticaria pigmentosa and aggressive mastocytosis: establishment of clonality in a human mast cell neoplasm
    B J Longley
    Department of Dermatology, Yale University School of Medicine, New Haven Connecticut 06510, USA
    Nat Genet 12:312-4. 1996
    b>Mastocytosis is characterized by accumulations of mast cells in various organs (1)...
  21. ncbi Establishment and characterization of a new canine mast cell tumor cell line
    T Ishiguro
    Department of Veterinary Clinical Sciences, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo, Japan
    J Vet Med Sci 63:1031-4. 2001
    ..This cell line will make an available source for studies on canine mast cell tumors...
  22. ncbi Abnormal expression of CD antigens in mastocytosis
    Luis Escribano
    Servicio de Hematologia, Mast Cell Unit, Hospital Ramon y Cajal, Madrid, Spain
    Int Arch Allergy Immunol 127:127-32. 2002
    ..Multiparametric flow-cytometric studies have shown that mast cells from mastocytosis display unique immunophenotypic characteristics, including coexpression of CD2 and CD25 antigens together with ..
  23. ncbi Association of paediatric mastocytosis with a polymorphism resulting in an amino acid substitution (M541L) in the transmembrane domain of c-KIT
    R Foster
    School of Biomedical Sciences, The University of Newcastle, Callaghan, New South Wales 2308, Australia
    Br J Dermatol 159:1160-9. 2008
    The receptor tyrosine kinase c-KIT plays a key role in normal mast cell development. Point mutations in c-KIT have been associated with sporadic or familial mastocytosis.
  24. ncbi Serum tryptase levels in patients with mastocytosis: correlation with mast cell burden and implication for defining the category of disease
    Wolfgang R Sperr
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Austria
    Int Arch Allergy Immunol 128:136-41. 2002
    The serum tryptase level is used as a diagnostic marker in mastocytosis and is considered to reflect the burden of (neoplastic) mast cells (MC).
  25. ncbi Mast cells and mastocytosis
    Johan D Soderholm
    Colorectal Surgery Unit, Department of Surgery, University Hospital, Linkoping, Sweden
    Dig Dis 27:129-36. 2009
    ..the tyrosine kinase- receptor for stem cell factor, are associated with the rare disease entity, systemic mastocytosis. These patients present symptoms arising from MC mediator release or infiltration...
  26. pmc PTEN deficiency in mast cells causes a mastocytosis-like proliferative disease that heightens allergic responses and vascular permeability
    Yasuko Furumoto
    Laboratory of Molecular Immunogenetics, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD 20892, USA
    Blood 118:5466-75. 2011
    ..The activating D816V mutation of Kit, seen in the majority of mastocytosis patients, causes a robust activation of PI3K signals...
  27. ncbi Enhanced protection against Heligmosomoides polygyrus in IL-2 receptor β-chain overexpressed transgenic mice with intestinal mastocytosis
    Masahiro Morimoto
    Laboratory of Veterinary Pathology, Faculty of Agriculture, Yamaguchi University, Japan
    J Vet Med Sci 73:849-51. 2011
    ..These mice also exhibit intestinal mastocytosis. Helminth infection induces various immune responses, such as mast cells, goblet cells, eosinophils and IgE, ..
  28. ncbi Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives
    Michel Arock
    Laboratoire de Biologie et Pharmacologie Appliquée, CNRS UMR 8113, Ecole Normale Superieure de Cachan, 61, Ave du Président Wilson, 94235 Cachan Cedex, France
    Expert Rev Hematol 3:497-516. 2010
    b>Mastocytosis is a myeloid neoplasm characterized by abnormal accumulation and frequent activation of mast cells (MCs) in various organs. Organ systems typically involved are the bone marrow, skin, liver and gastrointestinal tract...
  29. ncbi CD203c is overexpressed on neoplastic mast cells in systemic mastocytosis and is upregulated upon IgE receptor cross-linking
    A W Hauswirth
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria
    Int J Immunopathol Pharmacol 21:797-806. 2008
    ..expression of CD203c on normal mast cells (MC)and bone marrow (bm) MC derived from 85 patients with systemic mastocytosis (SM), including cases with indolent SM (ISM, n=72), SM with associated clonal hematologic non-MC-lineage ..
  30. ncbi Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria
    P Valent
    Department of Internal Medicine I, Division of Haematology and Haemostaseology, Medical University of Vienna, Vienna, Austria
    Eur J Clin Invest 37:435-53. 2007
    Although a classification for mastocytosis and diagnostic criteria are available, there remains a need to define standards for the application of diagnostic tests, clinical evaluations, and treatment responses...
  31. ncbi Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy
    B J Longley
    Department of Dermatology and Pathology, Columbia University, 630 West 168th Street, VC-15-202, New York, NY 10031, USA
    Leuk Res 25:571-6. 2001
    Mutations causing constitutive activation of KIT have been shown to be causative in some forms of mastocytosis, and several types of mutations have been associated with myeloproliferative disorders (MPDs), acute myelogenous leukemia (AML),..
  32. ncbi Mastocytosis: current concepts in diagnosis and treatment
    L Escribano
    Hospital Ramon y Cajal, Unidad de Mastocitosis, Carretera de Colmenar Km 9 1, Madrid 28034, Spain
    Ann Hematol 81:677-90. 2002
    b>Mastocytosis consists of a group of disorders characterized by a pathologic increase in mast cells in tissues including skin, bone marrow, liver, spleen, and lymph nodes. Mastocytosis is a rare disease...
  33. pmc Pulmonary mastocytosis and enhanced lung inflammation in mice heterozygous null for the Foxf1 gene
    Tanya V Kalin
    Division of Pulmonary Biology, Cincinnati Children s Hospital Research Foundation, Cincinnati, OH 45229, USA
    Am J Respir Cell Mol Biol 39:390-9. 2008
    ..Foxf1 haploinsufficiency caused pulmonary mastocytosis and enhanced pulmonary inflammation after chemically induced or allergen-mediated lung injury, indicating an ..
  34. ncbi Localized eosinophilic ileitis with mastocytosis successfully treated with oral budesonide
    C Lombardi
    Allergy and Respiratory Diseases, Department of Internal Medicine, Genoa, Italy
    Allergy 62:1343-5. 2007
  35. ncbi Ichthyosis congenita type IV: a new case resembling diffuse cutaneous mastocytosis
    A Brusasco
    Centre for Inherited Cutaneous Diseases, IRCCS, Ospedale Maggiore, University of Milan, Italy
    Br J Dermatol 136:377-9. 1997
    ..was further complicated by hypereosinophilia and a strongly positive Darier's sign suggesting diffuse cutaneous mastocytosis. The diagnosis was provided only by electron microscopy, which showed the pathognomonic markers of ichthyosis ..
  36. ncbi Mastocytosis: pathology, genetics, and current options for therapy
    Peter Valent
    Department of Internal Medicine I, Medical Univeristy of Vienna, Austria
    Leuk Lymphoma 46:35-48. 2005
    ..Symptoms in mastocytosis result from MC-derived mediators and, less frequently, from destructive infiltration of MCs...
  37. ncbi Comparative analysis of mutation of tyrosine kinase kit in mast cells from patients with systemic mast cell activation syndrome and healthy subjects
    Gerhard J Molderings
    Institute of Human Genetics, University Hospital of Bonn, Sigmund Freud Strasse 25, Bonn, Germany
    Immunogenetics 62:721-7. 2010
    ..The present findings support the idea that the systemic mast cell activation syndrome is a clonal disease most commonly associated with variable activating mutations in the tyrosine kinase kit...
  38. ncbi Diagnostic value of tryptase in anaphylaxis and mastocytosis
    Lawrence B Schwartz
    Division of Rheumatology, Allergy, and Immunology, Department of Internal Medicine, Virginia Commonwealth University, PO Box 980263, McGuire Hall 4 110, Richmond, VA 23298, USA
    Immunol Allergy Clin North Am 26:451-63. 2006
    ..mature tryptase measurements are recommended in the diagnostic evaluation of systemic anaphylaxis and systemic mastocytosis, but their interpretation must be considered in the context of a complete workup of each patient...
  39. ncbi RasGRP4, a new mast cell-restricted Ras guanine nucleotide-releasing protein with calcium- and diacylglycerol-binding motifs. Identification of defective variants of this signaling protein in asthma, mastocytosis, and mast cell leukemia patients and demon
    Yi Yang
    Department of Medicine, Brigham and Women s Hospital, and Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 277:25756-74. 2002
    ..Transcript analysis of an asthma patient, a mastocytosis patient, and the HMC-1 cell line derived from a MC leukemia patient revealed the presence of substantial ..
  40. ncbi Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings
    H P Horny
    Institute of Pathology, Medical University of Lubeck, Ratzeburger Allee 160, D 23538 Lubeck, Germany
    Leuk Res 25:543-51. 2001
    ..in mast cell (MC) numbers in hemopoietic tissues may be associated with (a) primary neoplastic MC disease (mastocytosis); (b) non-mast cell lineage myelogenous disorders (myelodysplastic or myeloproliferative syndromes and myeloid ..
  41. ncbi Multiple novel alterations in Kit tyrosine kinase in patients with gastrointestinally pronounced systemic mast cell activation disorder
    Gerhard J Molderings
    Institut fur Pharmakologie und Toxikologie, Universitatsklinikum Bonn, Germany
    Scand J Gastroenterol 42:1045-53. 2007
    ....
  42. ncbi Mastocytosis: an unusual clonal disorder of bone marrow-derived hematopoietic progenitor cells
    Hans Peter Horny
    Institute of Pathology, D 91522 Ansbach, Germany
    Am J Clin Pathol 132:438-47. 2009
    b>Mastocytosis, an unusual disorder of bone marrow-derived, clonally transformed hematopoietic progenitor cells, exhibits a broad spectrum of clinical and morphologic features ranging from a self-limiting benign disorder (ie, juvenile ..
  43. ncbi E3 ligase-defective Cbl mutants lead to a generalized mastocytosis and myeloproliferative disease
    Srinivasa Rao Bandi
    Department of Medicine, Hematology and Oncology and the Interdisciplinary Center for Clinical Research, University Hospital Muenster, Muenster, Germany
    Blood 114:4197-208. 2009
    ..murine bone marrow retrovirally transduced with c-Cbl mutants and transplanted into mice led to a generalized mastocytosis, a myeloproliferative disease, and myeloid leukemia...
  44. ncbi Gain-of-function mutations in the extracellular domain of KIT are common in canine mast cell tumors
    Sébastien Letard
    Institut National de la Sante et de la Recherche Medicale, U891, Centre de Recherche en Cancerologie de Marseille, Molecular and Functional Hematopoiesis, Marseille F 13009, France
    Mol Cancer Res 6:1137-45. 2008
    ..These results also show that Ba/F3 cells can be used for the direct characterization of canine KIT mutants, eliminating the need to make equivalent mutations in the mouse or human genes...
  45. pmc Mast cells and mastocytosis
    Dean D Metcalfe
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
    Blood 112:946-56. 2008
    ..In hematology, the clinical disease of mastocytosis is characterized by a pathologic increase of mast cells in tissues, often associated with mutations in KIT, the ..
  46. pmc Evidence for cognitive impairment in mastocytosis: prevalence, features and correlations to depression
    Daniela Silva Moura
    centre de référence des mastocytoses, Hopital Necker Enfants Malades, Fondation Imagine Paris, Universite Paris Descartes, Sorbonne, Paris Cité, Paris, France
    PLoS ONE 7:e39468. 2012
    b>Mastocytosis is a heterogeneous disease characterized by mast cells accumulation in one or more organs...
  47. ncbi Gene expression analysis in mastocytosis reveals a highly consistent profile with candidate molecular markers
    Claudio D'Ambrosio
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases NIAID, Bethesda, MD 20892 1881, USA
    J Allergy Clin Immunol 112:1162-70. 2003
    b>Mastocytosis is a rare clonal disorder that might be accompanied by non-mast-cell clonal hematologic disorders, such as myeloproliferative or myelodysplastic syndromes.
  48. pmc Chymase expressing bone marrow mast cells in mastocytosis and myelodysplastic syndromes: an immunohistochemical and morphometric study
    H P Horny
    Institute of Pathology, University of Lubeck, Ratzeburger Allee 160, D 23538 Lubeck, Germany
    J Clin Pathol 56:103-6. 2003
    ..Tryptase is constitutively expressed by all MC, whereas chymase is found only in an MC subset. Very little is known about chymase expression in MC proliferative disorders (mastocytosis).
  49. pmc Effects of anti-allergic drugs on intestinal mastocytosis and worm expulsion of rats infected with Neodiplostomum seoulense
    Eun Hee Shin
    Department of Parasitology and Tropical Medicine, College of Medicine and Institute of Endemic Diseases, Medical Research Center, Seoul National University, Seoul 110 799, Korea
    Korean J Parasitol 41:81-7. 2003
    The effects of anti-allergic drugs on intestinal mastocytosis and the expulsion of Neodiplostomum seoulense were observed in Sprague-Dawley rats, after oral infection with 500 metacercariae...
  50. pmc Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology
    Ashraf Uzzaman
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
    Pediatr Blood Cancer 53:629-34. 2009
    Pediatric onset mastocytosis usually presents as urticaria pigmentosa; and less often as diffuse cutaneous mastocytosis...
  51. ncbi [Mastocytosis. A challenge in anaesthesiology]
    F M Konrad
    Klinik für Anaesthesiologie und Intensivmedizin, Universitatsklinikum Tubingen
    Anaesthesist 58:1239-43. 2009
    b>Mastocytosis is a general term for a heterogeneous group of rare disorders. Many agents used in anaesthesia can trigger mast cell degranulation with release of histamine, prostaglandin, tryptase and heparin...
  52. doi High omalizumab dose controls recurrent reactions to venom immunotherapy in indolent systemic mastocytosis
    K Kontou-Fili
    Chief Allergology Department, Euroclinic Hospital, Athens, 8 Fidippidou st, Athens 11526, Hellas, Greece
    Allergy 63:376-8. 2008
  53. ncbi Kit: molecule of interest for the diagnosis and treatment of mastocytosis and other neoplastic disorders
    Mrinal M Patnaik
    Department of Internal Medicine, University of Minnesota, Minneapolis, MN 55455, USA
    Curr Cancer Drug Targets 7:492-503. 2007
    ..found to be an important component of oncogenesis in a large number of neoplastic disorders such as systemic mastocytosis, gastro intestinal stromal tumors, germ cell tumors, acute myelogenous leukemia with the disruption of the core ..
  54. pmc Phenotypic and genotypic characteristics of mastocytosis according to the age of onset
    Fanny Lanternier
    Universite Paris V, Service de Maladies Infectieuses et Tropicales, centre de référence des mastocytoses, Hopital Necker Enfants Malades, Centre d Infectiologie Necker Pasteur, Paris, France
    PLoS ONE 3:e1906. 2008
    Adult's mastocytosis is usually associated with persistent systemic involvement and c-kit 816 mutation, while pediatrics disease is mostly limited to the skin and often resolves spontaneously...
  55. ncbi A critical reappraisal of treatment response criteria in systemic mastocytosis and a proposal for revisions
    A Pardanani
    Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Eur J Haematol 84:371-8. 2010
    ..In adults, there is almost always MC infiltration of the bone marrow, which is a cardinal feature of systemic mastocytosis (SM)...
  56. ncbi Current options in the treatment of mast cell mediator-related symptoms in mastocytosis
    Luis Escribano
    Unidad de Mastocitosis, Laboratorio K Frank Austen, Hospital Ramon y Cajal, Red Española de Mastocitosis, Madrid, Spain
    Inflamm Allergy Drug Targets 5:61-77. 2006
    Patients with mastocytosis have symptoms related to the tissue response to the release of mediators from mast cells (MC), local mast cell burden or associated non-mast cell hematological disorders...
  57. pmc Regulation of normal and neoplastic human mast cell development in mastocytosis
    Dean D Metcalfe
    NIH NIAID LAD, Building 10, Room 11C205, 10 Center Drive, MSC 1881, Bethesda, MD 20892 1881, USA
    Trans Am Clin Climatol Assoc 116:185-203; discussion 203-4. 2005
    b>Mastocytosis, a condition characterized by a pathologic accumulation of clonal mast cells in tissues, offers a unique opportunity to study the growth and differentiation of mast cells as well as their contribution to various pathologic ..
  58. ncbi Establishment of a murine model of aggressive systemic mastocytosis/mast cell leukemia
    Shadmehr Demehri
    Oregon Health and Science University Cancer Institute, Portland, OR 97239, USA
    Exp Hematol 34:284-8. 2006
    Aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL) are rare but devastating diseases, for which no effective specific therapy is currently available...
  59. ncbi Mastocytosis - an update
    Ulrich Amon
    PsoriSol Clinic for Dermatology, Hersbruck, Germany
    J Dtsch Dermatol Ges 8:695-711; quiz 712. 2010
    b>Mastocytosis (MC) encompasses a range of disorders characterized by a clonal, pathological accumulation of mast cells having a somatic activating mutation of the tyrosine kinase receptor Kit (exon 17, codon 816; D816V) in more than 90 % ..
  60. ncbi Stinging Hymenoptera and mastocytosis
    Franziska Rueff
    AllergieZentrum, Klinik und Poliklinik fur Dermatologie und Allergologie, Ludwig Maximilians Universitat, Frauenlobstrasse 9 11, Munchen D 80337, Germany
    Curr Opin Allergy Clin Immunol 9:338-42. 2009
    Patients suffering from mastocytosis are at risk for a particularly severe Hymenoptera sting anaphylaxis...
  61. ncbi Elevated tryptase levels are associated with greater bone density in a cohort of patients with mastocytosis
    Nataliya M Kushnir-Sukhov
    Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, MD 20892 1881, USA
    Int Arch Allergy Immunol 139:265-70. 2006
    b>Mastocytosis is associated with a pathological increase in tissue mast cells. Associated skeletal problems include a decrease in bone density and pathological fractures.
  62. pmc Increased angiogenesis in the bone marrow of patients with systemic mastocytosis
    Friedrich Wimazal
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Vienna, Austria
    Am J Pathol 160:1639-45. 2002
    ..Systemic mastocytosis (SM) is a neoplasm affecting multilineage and mast cell (MC)-committed hemopoietic progenitors...
  63. pmc Intestinal protection against Strongyloides ratti and mastocytosis induced by administration of interleukin-3 in mice
    T Abe
    Department of Parasitology, Akita University School of Medicine, Japan
    Immunology 80:116-21. 1993
    ..The IL-3 treatment induced no protection or mastocytosis in mast cell-deficient W/Wv mice. These results suggest that the IL-3-induced intestinal protection against S...
  64. ncbi Mastocytosis: molecular mechanisms and clinical disease heterogeneity
    D D Metcalfe
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bldg. 10, Rm. 11C205, 10 Center Drive MSC 1881, Bethesda, MD 20892-1881, USA
    Leuk Res 25:577-82. 2001
    Systemic mastocytosis has one unifying feature: an unexplained and pathologic increase in mast cells in specific tissues...
  65. pmc The alpha form of human tryptase is the predominant type present in blood at baseline in normal subjects and is elevated in those with systemic mastocytosis
    L B Schwartz
    Department of Medicine, Virginia Commonwealth University, Richmond 23298, USA
    J Clin Invest 96:2702-10. 1995
    Tryptase, a protease produced by all mast cells, was evaluated as a clinical marker of systemic mastocytosis. Two sandwich immunoassays were evaluated, one which used the mAb G5 for capture, the other which used B12 for capture...
  66. ncbi Mastocytosis and fibrosis: role of cytokines
    Chin Yang Li
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA
    Int Arch Allergy Immunol 127:123-6. 2002
    b>Mastocytosis is a rare stem cell disorder characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Clinical heterogeneity is a hallmark of mastocytosis...
  67. ncbi Levels of mast-cell growth factors in plasma and in suction skin blister fluid in adults with mastocytosis: correlation with dermal mast-cell numbers and mast-cell tryptase
    Knut Brockow
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
    J Allergy Clin Immunol 109:82-8. 2002
    Mast-cell accumulation has been observed in the skin and other organs of patients with systemic indolent mastocytosis (SM). The basis for this pathologic increase is not fully understood.
  68. ncbi Determination of 1-methylhistamine and 1-methylimidazoleacetic acid in human urine as a tool for the diagnosis of mastocytosis
    J Martens-Lobenhoffer
    Institute of Clinical Pharmacology, Otto von Guericke University Magdeburg, Germany
    J Chromatogr B Biomed Sci Appl 721:135-40. 1999
    ..MHIS) and 1-methylimidazoleacetic acid (MIIA), in human urine is a useful tool for the diagnosis of mastocytosis. MHIS was extracted under basic conditions with chloroform and derivatized with trifluoroacetic acid anhydride, ..
  69. ncbi Complex karyotype and absence of mutation in the c-kit receptor in aggressive mastocytosis presenting with pelvic osteolysis, eosinophilia and brain damage
    E Jost
    Department of Hematology, Universite Catholique de Louvain, Bruxelles, Belgium
    Ann Hematol 80:302-7. 2001
    Aggressive mastocytosis is a form of systemic mast cell disease (SMCD) characterized by organ infiltration, bone lesions. eosinophilia and lymphadenopathies...
  70. ncbi Class III receptor tyrosine kinases: role in leukaemogenesis
    John T Reilly
    Molecular Haematology Unit, Division of Molecular and Genetic Medicine, Royal Hallamshire Hospital, Sheffield, UK
    Br J Haematol 116:744-57. 2002
  71. pmc Depression in patients with mastocytosis: prevalence, features and effects of masitinib therapy
    Daniela Silva Moura
    Universite Paris Descartes, Sorbonne, Paris Cité, Service d Hematologie, centre de référence des mastocytoses, Hopital Necker Enfants Malades, Paris, France
    PLoS ONE 6:e26375. 2011
    Depression in patients with mastocytosis is often reported but its prevalence and characteristics are not precisely described...
  72. ncbi Clinical and histopathological aspects of cutaneous mastocytosis
    K Wolff
    Department of Dermatology, Division of General Dermatology, University of Vienna, Vienna General Hospital, Waehringer Guertel 18 20, 1090 Vienna, Austria
    Leuk Res 25:519-28. 2001
    The organ most frequently involved in mastocytosis is the skin. Cutaneous mastocytosis (CM) is classified according to clinical presentation and is further defined by onset of disease...
  73. ncbi Treatment of cutaneous mastocytosis
    Klaus Wolff
    Department of Dermatology, University of Vienna, Austria
    Int Arch Allergy Immunol 127:156-9. 2002
    Therapy of cutaneous mastocytosis is directed towards skin and systemic symptoms due to mediator release and targeted on skin lesions...
  74. ncbi Evidence for altered mast cell proliferation and apoptosis in cutaneous mastocytosis
    K Hartmann
    Departments of Dermatology, University of Cologne, Joseph Stelzmann Str 9, 50931 Cologne, Germany
    Br J Dermatol 149:554-9. 2003
    b>Mastocytosis presents as a focal or generalized increase of mast cells, particularly in the skin, but also in other organs...
  75. ncbi Effect of granulocyte macrophage colony-stimulating factor in a patient with benign systemic mastocytosis
    T Zuberbier
    Department of Dermatology and Allergy, Charite, Humboldt University, 13344 Berlin, Germany
    Br J Dermatol 145:661-6. 2001
    ..a known inhibitor of in vitro mast cell differentiation, in a patient with benign, adult-onset systemic mastocytosis. In vitro effects of GM-CSF on bone marrow cultures before the start of treatment showed a marked inhibition of ..
  76. pmc Paediatric mastocytosis
    M C Carter
    NIAID NIH, Bethesda, MD 20892 1881, USA
    Arch Dis Child 86:315-9. 2002
  77. ncbi Bullous mastocytosis: a fatal outcome
    M Murphy
    Department of Dermatology, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland
    Pediatr Dermatol 16:452-5. 1999
    A 6-week-old boy was referred with a generalized bullous rash since birth. Examination revealed bullous mastocytosis with initially no evidence of systemic involvement...
  78. ncbi Cutaneous mastocytosis -- clinical heterogeneity
    Karin Hartmann
    Department of Dermatology, University Hospital, Cologne, Germany
    Int Arch Allergy Immunol 127:143-6. 2002
    Cutaneous mastocytosis (CM) encompasses several distinct patterns of disease which can be distinguished by the type of skin lesions, age of onset, familial occurrence, noncutaneous involvement, associated c-kit mutations, and prognosis...
  79. doi Allergen specific immunotherapy is safe and effective in patients with systemic mastocytosis and Hymenoptera allergy
    Patrizia Bonadonna
    J Allergy Clin Immunol 121:256-7. 2008
  80. ncbi How much specific is the association between hymenoptera venom allergy and mastocytosis?
    P Bonadonna
    Verona General Hospital, Italy
    Allergy 64:1379-82. 2009
    The preferential association of mastocytosis with hymenoptera sting reactions is well known, but there is no data on the prevalence of clonal mast cell disorders in subjects with severe systemic reactions due to foods or drugs.
  81. ncbi Clinical scoring of cutaneous mastocytosis
    R Heide
    Department of Dermato-Venereology, University Hospital Rotterdam, The Netherlands
    Acta Derm Venereol 81:273-6. 2001
    There are still many controversies in defining and evaluating mastocytosis. One of the aspects that is missing is a system for clinical evaluation of mastocytosis of the skin...
  82. ncbi Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report
    L M Waxtein
    Department of Dermatology, , Mexico City, Mexico
    Pediatr Dermatol 17:198-201. 2000
    b>Mastocytosis encompasses a range of disorders characterized by overproliferation and accumulation of tissue mast cells...
  83. ncbi Presentation of cutaneous mastocytosis in 173 children
    R Hannaford
    New Children s Hospital, Sydney, New South Wales, Australia
    Australas J Dermatol 42:15-21. 2001
    ..is a retrospective review of the case files and clinical photographs of 173 children diagnosed with cutaneous mastocytosis by a dermatologist in an exclusively paediatric practice...
  84. ncbi Neuropeptide blood levels correlate with mast cell load in patients with mastocytosis
    L Maintz
    Department of Dermatology and Allergy, University of Bonn, Bonn, Germany
    Allergy 66:862-9. 2011
    b>Mastocytosis is characterized by abnormal growth and accumulation of mast cells (MCs) in different organs. MCs have been shown to express receptors for neuropeptides...
  85. ncbi Mastocytosis: mediator-related signs and symptoms
    Mariana Castells
    Brigham and Women s Hospital and Harvard Medical School, Boston, MA 02115, USA
    Int Arch Allergy Immunol 127:147-52. 2002
    Patients with systemic mastocytosis present symptoms related to the tissue response to the release of mediators from mast cells and to the local mast cell burden...
  86. pmc Raised tryptase without anaphylaxis or mastocytosis: heterophilic antibody interference in the serum tryptase assay
    R Sargur
    Department of Immunology, Northern General Hospital, Sheffield, UK
    Clin Exp Immunol 163:339-45. 2011
    Mast cell tryptase (MCT) is a key diagnostic test for mastocytosis and anaphylaxis...
  87. ncbi Mastocytosis and allergy
    Matthew Greenhawt
    Division of Allergy and Immunology, Department of Internal Medicine, University of Michigan, School of Medicine, Ann Arbor, Michigan 48109, USA
    Curr Opin Allergy Clin Immunol 7:387-92. 2007
    To illustrate features of allergy in mastocytosis.
  88. ncbi Mastocytosis: recent advances in defining the disease
    K Hartmann
    Department of Dermatology, University of Cologne, Joseph-Stelzmann-Str. 9, 50931 Cologne, Germany
    Br J Dermatol 144:682-95. 2001
    b>Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues, which may present in a variety of clinical patterns...
  89. ncbi Histopathological and immunohistochemical aspects of mastocytosis
    Hans Peter Horny
    Institute of Pathology, Medical University of Lubeck, Germany
    Int Arch Allergy Immunol 127:115-7. 2002
    The diagnosis of mastocytosis is based on histological evidence of a focal increase in tissue mast cells...
  90. ncbi Parkinsonism due to bilateral basal ganglia lesions following mastocytosis-induced hypoxia
    Axel Schramm
    J Neurol 251:1270-2. 2004
  91. ncbi [Systemic mastocytosis--definition of an internal disease]
    Jürgen Homann
    Abteilung fur Innere Medizin, Evangelisches Waldkrankenhaus, Bonn, Germany
    Med Klin (Munich) 105:544-53. 2010
    Systemic mastocytosis comprises disorders characterized by an accumulation of genetically altered mast cells in all organs and tissues due to an increased proliferation rate and reduced apoptosis of those pathologic mast cells...
  92. ncbi Human mast cells and mastocytosis: harnessing microRNA expression as a new approach to therapy?
    Lorenzo Deho'
    Institute for Research in Biomedicine, Via Vincenzo Vela 6, 6500 Bellinzona, Switzerland
    Arch Immunol Ther Exp (Warsz) 58:279-86. 2010
    ....
  93. ncbi Mastocytosis and insect venom allergy
    Patrizia Bonadonna
    Allergy Unit, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy
    Curr Opin Allergy Clin Immunol 10:347-53. 2010
    To analyse the association of systemic allergic hymenoptera sting reactions with mastocytosis and elevated baseline serum tryptase and to discuss diagnosis and treatment in patients with both diseases.
  94. ncbi Pathogenesis, clinical features, and treatment advances in mastocytosis
    A Pardanani
    Division of Hematology, Mayo Clinic, Mayo Building W10A, 200 First Street SW, Rochester, MN 55905, USA
    Best Pract Res Clin Haematol 19:595-615. 2006
    Systemic mastocytosis (SM) is characterized by the abnormal growth and accumulation of mast cells (MC) in one or more organs...
  95. ncbi KIT and mastocytosis
    Ken Hong Lim
    Division of Hematology, Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    Acta Haematol 119:194-8. 2008
    ..been associated with myeloid malignancies including core binding factor acute myeloid leukemia and systemic mastocytosis (SM), germ cell tumors, gastrointestinal stromal tumors and sinonasal T cell lymphomas...
  96. ncbi Soluble stem cell factor receptor (CD117) and IL-2 receptor alpha chain (CD25) levels in the plasma of patients with mastocytosis: relationships to disease severity and bone marrow pathology
    C Akin
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
    Blood 96:1267-73. 2000
    Systemic mastocytosis is a disease of mast cell proliferation that may be associated with hematologic disorders...
  97. ncbi Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA)
    D Gonzalez de Olano
    Allergy Department, Hospital Ramon y Cajal, Madrid, Spain
    Clin Exp Allergy 37:1547-55. 2007
    ..b>Mastocytosis is characterized by an abnormal proliferation and accumulation of mast cells, in which mediators are released ..
  98. ncbi Systemic mastocytosis with plasma cell dyscrasia: report of a case
    Sheeja T Pullarkat
    Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, 10833 Le Conte Avenue, Los Angeles, CA 90095, United States
    Leuk Res 32:1160-3. 2008
    Systemic mastocytosis (SM) comprises a heterogeneous group of disorders characterized by infiltration of bone marrow and other tissues by neoplastic mast cells...
  99. ncbi Analysis of the surface expression of c-kit and occurrence of the c-kit Asp816Val activating mutation in T cells, B cells, and myelomonocytic cells in patients with mastocytosis
    C Akin
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1881, USA
    Exp Hematol 28:140-7. 2000
    The Asp816Val c-kit activating mutation is detectable in the peripheral blood cells of some patients with mastocytosis and in lesional skin biopsies obtained from adult patients with urticaria pigmentosa...
  100. ncbi Mastocytosis and Hymenoptera allergy
    Anthony E J Dubois
    Department of Allergology, University Hospital Groningen, Groningen, The Netherlands
    Curr Opin Allergy Clin Immunol 4:291-5. 2004
    b>Mastocytosis is a rare disease with associations with anaphylactic reactions to Hymenoptera stings...
  101. ncbi Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease
    Knut Brockow
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892 1881, USA
    Arch Dermatol 138:785-90. 2002
    To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM).

Research Grants41

  1. Targeting Siglec-8/-F to treat eosinophil and mast cell related disorders
    Bruce S Bochner; Fiscal Year: 2012
    ..Systemic Mastocytosis, a malignant disease, presents with varying prognoses depending on the extent of involvement, but Aggressive ..
  2. Targeting Siglec-8/-F to treat eosinophil and mast cell related disorders
    Bruce S Bochner; Fiscal Year: 2013
    ..Systemic Mastocytosis, a malignant disease, presents with varying prognoses depending on the extent of involvement, but Aggressive ..
  3. System for Rapid Generation of Aptamers for Transplant Therapies
    KAREN QIAN; Fiscal Year: 2010
    ..and solid tumors such as acute myeloid leukemia, myelodysplastic syndromes, chronic myeloid leukemia, systemic mastocytosis and breast cancer...
  4. Oncogenic Kit receptor signaling in vivo
    Peter Besmer; Fiscal Year: 2012
    ..In human neoplasia oncogenic activation of Kit has roles in gastrointestinal stromal tumors (GIST), mastocytosis/mast cell leukemia, acute myelogenous leukemia, a subset of melanomas and a subset of germ cell tumors...
  5. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2013
    ..gastrointestinal stromal tumors (GISTs), sinonasal lymphomas, acute myeloid leukemia (AML), and systemic mastocytosis (SM). A significant proportion of these diseases commonly bear the KIT activation loop mutation KITD816V...
  6. Trefoil Factors Regulate Th2 Immunity
    DEBROSKI R HERBERT; Fiscal Year: 2013
    ..by production of interleukins (IL's) 4, 5, 13, 25, and IL-33, immunoglobulin E (IgE) eosinophilia, mastocytosis, basophilia, alternatively activated macrophages (AAM), smooth muscle hypercontractility, and mucus ..
  7. A Mouse Model for Human Gastrointestinal Stromal Tumor
    Peter Besmer; Fiscal Year: 2013
    ..In human neoplasia oncogenic activation of Kit has roles in gastrointestinal stromal tumors, mastocytosis/mast cell leukemia, acute myelogenous leukemia, a minor subset of melanomas and a subset of germ cell tumors...
  8. Novel Mechanisms of C-Kit Regulation in Mast Cells
    Reuben Kapur; Fiscal Year: 2013
    ..b>Mastocytosis, especially the systemic form of the disease, also known as systemic mastocytosis (SM) is considered a ..
  9. Mechanisms of Tyrosine Kinase Inhibitor Resistance in GI Stromal Tumors
    Michael Heinrich; Fiscal Year: 2012
    ..g. melanoma, acute myeloid leukemia, seminoma, and mastocytosis)...
  10. CysLT and P2Y Receptors in Lung Inflammation
    Joshua A Boyce; Fiscal Year: 2012
    ..preliminary studies now demonstrate that cys-LTs play an unanticipated, essential role in mediating reactive mastocytosis in a model of allergen-induced pulmonary inflammation...
  11. GROWTH AND DIFFERENTIATION OF HUMAN MAST CELLS
    ROBERT BRESSLER; Fiscal Year: 1993
    ..more similar to basophils than mast cells, whereas HCGC derived from bone marrow of a patient with systemic mastocytosis (SM), were more characteristic of mast cells...
  12. IMMUNOLOGIC RELEASE OF CHEMICAL MEDIATORS IN MAN
    FRANK AUSTEN; Fiscal Year: 1990
    ..Mast cells will be defined in situ in patients with systemic mastocytosis and chemically/immunochemically in biopsy tissues to seek differences which might be highlighted by a ..
  13. DIFFERENT IMBALANCES OF CYTOKINES IN NEMATODE INFECTIONS
    Norman Reed; Fiscal Year: 1992
    ..Nippostrongylus brasiliensis (Nb) and Trichinella spiralis (Ts) cause striking intestinal mastocytosis; by contrast, Nematospiroides dubius (Nd) not only fails to cause mastocytosis but also inhibits the ..
  14. SECRETION OF BLADDER MAST CELL MEDIATORS IN IC
    Theoharis Theoharides; Fiscal Year: 1991
    ..IgE) and antigen (Ag) leading to increased secretion of inflammatory mediators in allergies, asthma, systemic mastocytosis and pruritus...
  15. Recruitment of Murine Mast Cell Progenitors
    JUAN ABONIA; Fiscal Year: 2007
    ..Additionally a significant intestinal mastocytosis is also noted in the challenged animals...
  16. PROSTANOID BIOSYNTHESIS IN SYSTEMIC MASTOCYTOSIS
    John Oates; Fiscal Year: 2003
    ..research is to examine the biosynthesis of the mast cell mediator, prostaglandin D2, in patients with systemic mastocytosis, and to explore the potential for improvements in the treatment of this disorder...
  17. CysLt and P2Y Receptors and Lung Inflammation
    Joshua Boyce; Fiscal Year: 2007
    ..of an autocrine/intracrine signaling loop that is essential for the expansion of MC numbers (reactive mastocytosis) occurring in the lung and intestinal mucosal surfaces in Th2-biased immune responses in vivo...
  18. BIOCHEMISTRY OF MAST CELL SECRETORY GRANULE ENZYMES
    Lawrence Schwartz; Fiscal Year: 2000
    ..used to assess mast cell involvement in human diseases such as asthma, systemic anaphylaxis and systemic mastocytosis, the biochemical characterization of the enzyme as a heparin-stabilized tetramer resistant to biologic protease ..
  19. CUTANEOUS BIOLOGY OF KIT LIGAND
    JACK LONGLEY; Fiscal Year: 2005
    ..The goal of this proposal is to determine the role of the SCF-KIT signaling pathway in mastocytosis and cutaneous inflammation...
  20. MICROPHTHALMIA--CRITICAL FACTOR IN MAST CELL DEVELOPMENT
    Clifford Takemoto; Fiscal Year: 2001
    ..In addition, mast cell hyperplasia results in disorders such as mastocytosis. Thus, mast cells are not only an important disease-specific cell lineage, but also provide an attractive ..
  21. Gene and Protein Defects Causing Mastocytosis
    Yongsheng Ma; Fiscal Year: 2007
    The goal of this proposal is to add to the understanding of the mechanisms underlying the pathogenesis of mastocytosis. They have previously shown that essentially all adult and atypical pediatric mastocytosis are characterized by ..
  22. Cutaneous Biology KIT Ligand
    JACK LONGLEY; Fiscal Year: 2007
    ..KIT controls MAGE gene promoter methylation, and will determine whether MAGE protein expression is common in mastocytosis by immunohistochemistry and reverse transcription-PCR...
  23. IL-4 INDEPENDENT IL-13 MEDIATED PROTECTIVE IMMUNITY
    William Gause; Fiscal Year: 2004
    ..against Trichuris infection has been shown to be T cell-dependent and associated with IgE elevations and mastocytosis. We have used the natural murine parasite, Trichuris muris, as a model for understanding the development of the ..
  24. Direct IL-4 and IL-13 effects on pulmonary smooth muscle in allergic airway disea
    FRED DOUGLASS FINKELMAN; Fiscal Year: 2010
    ..Proposed studies with a mouse model in which airway hyperresponsiveness is induced solely by direct IL-4 and IL-13 effects on smooth muscle should help to elucidate these mechanisms and may reveal new approaches for asthma therapy. ..
  25. IL4R AND IMMUNITY TO GI NEMATODE PARASITES
    Fred Finkelman; Fiscal Year: 2003
    ....
  26. Mast cells, antidepressants and chronic fatigue syndrome
    Theoharis Theoharides; Fiscal Year: 2007
    ..Future studies will build on these findings to develop in vitro and in vivo models of CFS and lead to clinical trials with select antidepressants or other molecules that inhibit brain mast cells. [unreadable] [unreadable] [unreadable]..
  27. FTI Biology & Treatment of Myeloproliferative Disorders
    Jason Gotlib; Fiscal Year: 2005
    ..If clinically efficacious, further studies of R115777 in MPDs will be warranted...
  28. Cellular targets of IL-4 and IL-13 in worm expulsion
    Fred Finkelman; Fiscal Year: 2007
    ..It should also be useful for the intelligent prediction of risks associated with agents that inhibit allergic inflammation. ..
  29. CORTICOTROPIN RELEASING HORMONE INDUCED DURA MAST CELL A
    Theoharis Theoharides; Fiscal Year: 2001
    ..They may also help explain some key events in the pathophysiology of migraines, the clinical symptoms of which are often precipitated by stress. ..
  30. REGULATION OF IL4 PRODUCTION IN VIVO
    Fred Finkelman; Fiscal Year: 2002
    ..The results of these experiments will provide a better understanding of how IL-4 responses are regulated in vivo; this understanding will enhance the ability to control disorders that are regulated by IL-4. ..
  31. Role of Inflammatory Cytokines in the Allergic Response
    Fred Finkelman; Fiscal Year: 2007
    ..abstract_text> ..
  32. Restraint stress-induced neurogenic bladder inflammation
    Theoharis Theoharides; Fiscal Year: 2006
    ..Symptoms of urinary frequency and pelvic pain commonly worsen perimenstrually and under stress in IC. Bladder mastocytosis with mast cell activation has been documented in IC...
  33. CYTOKINE REGULATION OF A MOUSE MODEL OF ASTHMA
    Fred Finkelman; Fiscal Year: 2003
    ..By clarifying the mechanisms by which cytokines induce pulmonary inflammation and airway responsiveness, the proposed studies will provide information important for rational therapy for asthma. ..
  34. EVALUATION OF BASOPHIL INVOLVEMENT IN HUMAN DISEASE
    Lawrence Schwartz; Fiscal Year: 2001
    ....
  35. THE ROLE OF MAST CELL STAT6 IN TH2 DIFFERENTIATION
    Melanie Sherman; Fiscal Year: 2004
    ....
  36. BIOCHEMISTRY OF MAST CELL SECRETORY GRANULE ENZYMES
    Lawrence Schwartz; Fiscal Year: 2005
    ..used to assess mast cell involvement in human diseases such as asthma, systemic anaphylaxis and systemic mastocytosis, the biochemical characterization of the enzyme as a heparin-stabilized tetramer resistant to biologic protease ..
  37. Clofarabine: Cytarabine Activation for Leukemia Response
    Stefan Faderl; Fiscal Year: 2004
    ..abstract_text> ..
  38. Mast Cells: Role in the Pathophysiology of Headache
    Dan Levy; Fiscal Year: 2007
    ..Results may help identify pharmacological targets that could lead to the development of novel analgesic drugs for the treatment of headaches and possibly other intractable pain syndromes. [unreadable] [unreadable] [unreadable]..
  39. Genetic networks that regulate the mast lineage
    Clifford Takemoto; Fiscal Year: 2009
    ..This proposal will provide insights into the mechanisms of lineage-selection during mast cell development and may lead to therapeutic targets for mast cell-related diseases. ..
  40. CHARACTERIZATION OF DIFFERENT TYPES OF HUMAN MAST CELLS
    Lawrence Schwartz; Fiscal Year: 2009
    ..abstract_text> ..
  41. INDUCTION OF ATHEROGENESIS BY COCAINE AND HIV INFECTION
    Guha Krishnaswamy; Fiscal Year: 2001
    ..abstract_text> ..