systemic mastocytosis

Summary

Summary: A group of disorders caused by the abnormal proliferation of MAST CELLS in a variety of extracutaneous tissues including bone marrow, liver, spleen, lymph nodes, and gastrointestinal tract. Systemic mastocytosis is commonly seen in adults. These diseases are categorized on the basis of clinical features, pathologic findings, and prognosis.

Top Publications

  1. ncbi [Systemic mastocytosis]
    O Fain
    Service de Medecine Interne, Hopital Jean Verdier AP HP, Universite Paris XIII, Bondy
    Presse Med 34:681-7. 2005
  2. ncbi Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial
    Helga J Droogendijk
    Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
    Cancer 107:345-51. 2006
  3. ncbi Systemic mastocytosis: current classification and novel therapeutic options
    David A Barbie
    Department of Medical Oncology Hematologic Malignancies at Dana Farber Cancer Institute and Massachusetts General Hospital, Boston, MA 02115, USA
    Clin Adv Hematol Oncol 4:768-75. 2006
  4. ncbi Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis
    Barbara J Bain
    Department of Haematology, St Mary s Hospital Campus, Imperial College Faculty of Medicine, St Mary s Hospital, London, United Kingdom
    Am J Hematol 77:82-5. 2004
  5. ncbi Dasatinib (BMS-354825) inhibits KITD816V, an imatinib-resistant activating mutation that triggers neoplastic growth in most patients with systemic mastocytosis
    Neil P Shah
    Division of Hematology Oncology, The David Geffen School of Medicine at University of California Los Angeles UCLA, CA, USA
    Blood 108:286-91. 2006
  6. doi Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis
    Srdan Verstovsek
    Leukemia Department, M D Anderson Cancer Center, Houston, Texas 77030, USA
    Clin Cancer Res 14:3906-15. 2008
  7. ncbi Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria
    Peter Valent
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Wahringer Gurtel 18 20, A 1090, Vienna, Austria
    Leuk Res 27:635-41. 2003
  8. ncbi Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis
    Hejin P Hahn
    Department of Pathology, Brigham and Women s Hospital, and Harvard Medical School, Boston, MA 02115, USA
    Am J Surg Pathol 31:1669-76. 2007
  9. pmc Expression of activated STAT5 in neoplastic mast cells in systemic mastocytosis: subcellular distribution and role of the transforming oncoprotein KIT D816V
    Christian Baumgartner
    Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria
    Am J Pathol 175:2416-29. 2009
  10. doi Systemic mastocytosis in adults: a review on prognosis and treatment based on 342 Mayo Clinic patients and current literature
    Animesh Pardanani
    Department of Medicine, Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA
    Curr Opin Hematol 17:125-32. 2010

Research Grants

  1. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2011
  2. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2010
  3. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2009
  4. Activating PTPN11 and c-kit Mutations in Myeloproliferative Disorder
    Rebecca Chan; Fiscal Year: 2007
  5. PROSTANOID BIOSYNTHESIS IN SYSTEMIC MASTOCYTOSIS
    John Oates; Fiscal Year: 2003
  6. BIOCHEMISTRY OF MAST CELL SECRETORY GRANULE ENZYMES
    Lawrence Schwartz; Fiscal Year: 2005
  7. FTI Biology & Treatment of Myeloproliferative Disorders
    Jason Gotlib; Fiscal Year: 2005
  8. Stress Induces Skin Mast Cell Activation & Vasodilation
    Theoharis Theoharides; Fiscal Year: 2005
  9. Brain mast cells and Chronic Fatigue Syndrome
    Theoharis C Theoharides; Fiscal Year: 2010
  10. Restraint stress-induced neurogenic bladder inflammation
    Theoharis Theoharides; Fiscal Year: 2006

Detail Information

Publications176 found, 100 shown here

  1. ncbi [Systemic mastocytosis]
    O Fain
    Service de Medecine Interne, Hopital Jean Verdier AP HP, Universite Paris XIII, Bondy
    Presse Med 34:681-7. 2005
    b>Systemic mastocytosis is characterized by abnormal mast cell proliferation in different organs...
  2. ncbi Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial
    Helga J Droogendijk
    Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
    Cancer 107:345-51. 2006
    ..Imatinib mesylate is a potent inhibitor of c-kit receptor tyrosine kinase activity. Therefore, the authors evaluated the efficacy and safety of imatinib mesylate as treatment for patients with systemic mastocytosis.
  3. ncbi Systemic mastocytosis: current classification and novel therapeutic options
    David A Barbie
    Department of Medical Oncology Hematologic Malignancies at Dana Farber Cancer Institute and Massachusetts General Hospital, Boston, MA 02115, USA
    Clin Adv Hematol Oncol 4:768-75. 2006
    ..Novel strategies designed to inhibit the growth of mast cells containing the c-KIT D816V mutations have shown success in vitro and may provide effective targeted therapy for this treatment-refractory disease...
  4. ncbi Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis
    Barbara J Bain
    Department of Haematology, St Mary s Hospital Campus, Imperial College Faculty of Medicine, St Mary s Hospital, London, United Kingdom
    Am J Hematol 77:82-5. 2004
    ..also been found in patients with hypereosinophilia and atypical bone marrow mast cells but whether this syndrome should be regarded as a variant of eosinophilic leukemia or as a variant of systemic mastocytosis remains to be established.
  5. ncbi Dasatinib (BMS-354825) inhibits KITD816V, an imatinib-resistant activating mutation that triggers neoplastic growth in most patients with systemic mastocytosis
    Neil P Shah
    Division of Hematology Oncology, The David Geffen School of Medicine at University of California Los Angeles UCLA, CA, USA
    Blood 108:286-91. 2006
    ..mutation is inherently resistant to imatinib and, to date, there remains no effective curative therapy for systemic mastocytosis associated with KITD816V...
  6. doi Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis
    Srdan Verstovsek
    Leukemia Department, M D Anderson Cancer Center, Houston, Texas 77030, USA
    Clin Cancer Res 14:3906-15. 2008
    ..characterization of Philadelphia chromosome-negative (Ph-) chronic myeloproliferative disorders, such as systemic mastocytosis (SM), has provided a clear rationale for investigating novel targeted therapies...
  7. ncbi Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria
    Peter Valent
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Wahringer Gurtel 18 20, A 1090, Vienna, Austria
    Leuk Res 27:635-41. 2003
    Aggressive systemic mastocytosis (ASM) is a clonal mast cell disease characterized by progressive growth of neoplastic cells in diverse organs leading to organopathy...
  8. ncbi Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis
    Hejin P Hahn
    Department of Pathology, Brigham and Women s Hospital, and Harvard Medical School, Boston, MA 02115, USA
    Am J Surg Pathol 31:1669-76. 2007
    b>Systemic mastocytosis (SM) is characterized by the accumulation of neoplastic mast cells in bone marrow and other organs...
  9. pmc Expression of activated STAT5 in neoplastic mast cells in systemic mastocytosis: subcellular distribution and role of the transforming oncoprotein KIT D816V
    Christian Baumgartner
    Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria
    Am J Pathol 175:2416-29. 2009
    ..We examined the expression of pSTAT5 in neoplastic mast cells in systemic mastocytosis and asked whether the disease-related oncoprotein KIT D816V is involved in STAT5 activation...
  10. doi Systemic mastocytosis in adults: a review on prognosis and treatment based on 342 Mayo Clinic patients and current literature
    Animesh Pardanani
    Department of Medicine, Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA
    Curr Opin Hematol 17:125-32. 2010
    b>Systemic mastocytosis is a neoplastic disease of mast cells that often harbors a KIT mutation and involves the bone marrow...
  11. doi Variable presence of KITD816V in clonal haematological non-mast cell lineage diseases associated with systemic mastocytosis (SM-AHNMD)
    Karl Sotlar
    Institute of Pathology, University of Munich, Germany
    J Pathol 220:586-95. 2010
    In a substantial number of patients with systemic mastocytosis (SM), an associated clonal haematological non-mast cell lineage disease (AHNMD) is detectable...
  12. doi Gastrointestinal involvement and manifestations in systemic mastocytosis
    Harry Sokol
    Department of Gastroenterology and Nutrition, Saint Antoine Hospital, Universite Paris VI, Faculte de Medecine, Assistance Publique Hopitaux de Paris, Paris, France
    Inflamm Bowel Dis 16:1247-53. 2010
    ..GI manifestations in mastocytosis are reviewed. Pathogenesis of GI symptoms in systemic mastocytosis and their treatment are critically discussed...
  13. ncbi Immunophenotyping of mast cells: a sensitive and specific diagnostic tool for systemic mastocytosis
    P L A van Daele
    Department of Internal Medicine, Erasmus Medical Centre, University Medical Center Rotterdam, Rotterdam, The Netherlands
    Neth J Med 67:142-6. 2009
    The diagnosis of systemic mastocytosis (SM) is based on a combination of major and minor criteria. Flow cytometric detection of aberrant expression of CD2 and/or CD25 on CD117-positive mast cells is one of the minor criteria used...
  14. doi Multimodal therapy for vertebral involvement of systemic mastocytosis
    Antonio Krüger
    Department of Trauma Surgery, Philipps University, Marburg, Germany
    Spine (Phila Pa 1976) 34:E626-8. 2009
    ..Case report and clinical discussion...
  15. ncbi Cladribine therapy for systemic mastocytosis
    Hanneke C Kluin-Nelemans
    Department of Hematology, University Hospital Groningen, Hanzeplein 1, PO Box 30001, NL 9700 RB Groningen, The Netherlands
    Blood 102:4270-6. 2003
    Patients with systemic mastocytosis (SM) can suffer from disabling symptoms related to mast cell mediator release or mast cell infiltration, requiring mast cell eradication...
  16. ncbi Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis
    Melody C Carter
    J Allergy Clin Immunol 119:1550-1. 2007
  17. pmc Systemic mastocytosis associated with chronic idiopathic myelofibrosis: a distinct subtype of systemic mastocytosis associated with a [corrected] clonal hematological non-mast [corrected] cell lineage disorder carrying the activating point mutations KITD8
    Karl Sotlar
    Institute of Pathology, University of Tubingen, Liebermeisterstrasse 8, D 72076 Tubingen, Germany
    J Mol Diagn 10:58-66. 2008
    In approximately 20 to 30% of patients with systemic mastocytosis (SM), an associated clonal hematological nonmast cell lineage disorder (AHNMD) is diagnosed...
  18. doi Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis
    Arturo Vega-Ruiz
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
    Leuk Res 33:1481-4. 2009
    ..within the kinase domain of the transmembrane receptor KIT is found in the great majority of patients with systemic mastocytosis (SM) and is attractive therapeutic target...
  19. pmc FIP1L1/PDGFR alpha-associated systemic mastocytosis
    Yoshiyuki Yamada
    Division of Allergy and Immunology, Gunma Children s Medical Center, Shibukawa, Gunma, Japan yamaday gcmc pref gunma jp
    Int Arch Allergy Immunol 152:101-5. 2010
    ..are characterized by clonal hypereosinophilia, multiple organ dysfunctions due to eosinophil infiltration, systemic mastocytosis (SM) and a dramatic response to treatment with imatinib mesylate...
  20. ncbi Systemic mastocytosis: a potential neurologic emergency
    G B Boncoraglio
    Department of Neurology, Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy
    Neurology 65:332-3. 2005
  21. doi Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis
    David González de Olano
    Allergy Unit, Hospital de Fuenlabrada, Madrid, Spain
    J Allergy Clin Immunol 121:519-26. 2008
    ..Venom immunotherapy (VIT) is a safe and effective way to treat patients with Hymenoptera anaphylaxis, but few studies have addressed its usefulness in patients with systemic mastocytosis.
  22. ncbi Systemic mastocytosis: classification, pathogenesis, diagnosis, and treatment
    Olga Bunimovich
    Department of Dermatology, State University of New York, Buffalo School of Medicine, USA
    Cutis 83:29-36. 2009
    ..A gain-of-function mutation in codon 816 of c-kit is frequently present in mast cells of patients with systemic mastocytosis (SM)...
  23. ncbi Interferon treatment for hypereosinophilic syndromes and systemic mastocytosis
    J H Butterfield
    Divisions of Allergy and Immunology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Acta Haematol 114:26-40. 2005
    Hypereosinophilic syndromes (HES) and systemic mastocytosis (SMCD) are heterogeneous disorders with clinical symptoms from local and remote effects of excessive proliferation of eosinophils and mast cells, respectively...
  24. doi Systemic mastocytosis and bone involvement in a cohort of 75 patients
    S Barete
    AP HP, Dermatology and Allergy Department, Universite Pierre et Marie Curie, Tenon Hospital, 4 rue Chine, Paris 75020, France
    Ann Rheum Dis 69:1838-41. 2010
    To investigate bone involvement in a large cohort of systemic mastocytosis (SM) patients, and evaluate the efficacy of bisphosphonate therapy. Patients and
  25. doi Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine
    Ken H Lim
    Divisions of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Am J Hematol 84:790-4. 2009
    Cytoreductive therapy in systemic mastocytosis (SM) includes several drugs whose individual merit has not been well characterized...
  26. ncbi CD25 expression on cutaneous mast cells from adult patients presenting with urticaria pigmentosa is predictive of systemic mastocytosis
    Travis J Hollmann
    Department of Pathology, Brigham and Women s Hospital, Boston, MA 02115, USA
    Am J Surg Pathol 32:139-45. 2008
    ..proportion (up to 30%) of adolescent and adult-onset UP represents cutaneous involvement by underlying systemic mastocytosis (SM)...
  27. doi Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies
    Animesh Pardanani
    Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA
    Blood 114:3769-72. 2009
    The prognostic heterogeneity of the World Health Organization category of "systemic mastocytosis with associated clonal hematologic nonmast cell lineage disease" (SM-AHNMD) has not been systematically validated by primary data...
  28. ncbi Systemic mastocytosis
    Peter Valent
    Medical University of Vienna, Austria
    Cancer Treat Res 142:399-419. 2008
  29. pmc Genetic and pharmacologic evidence implicating the p85 alpha, but not p85 beta, regulatory subunit of PI3K and Rac2 GTPase in regulating oncogenic KIT-induced transformation in acute myeloid leukemia and systemic mastocytosis
    Veerendra Munugalavadla
    Department of Pediatrics, Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    Blood 110:1612-20. 2007
    Oncogenic activation loop KIT mutations are observed in acute myeloid leukemia (AML) and systemic mastocytosis (SM); however, unlike the KIT juxtamembrane mutants, the activation loop mutants are insensitive to imatinib mesylate...
  30. doi Ureteral stones due to systemic mastocytosis: diagnostic and therapeutic characteristics
    Gerhard J Molderings
    Institute of Human Genetics, University Hospital of Bonn, Bonn, Germany
    Urol Res 37:227-9. 2009
    Urolithiasis is expected to cause a considerable complication in patients with systemic mastocytosis. The aim of the present report is to demonstrate that due to pathological activation and irritability of mast cells, special features in ..
  31. ncbi Distinct clonal origins of systemic mastocytosis and associated B-cell lymphoma
    Young Kim
    Division of Pathology, City of Hope National Medical Center, 1500 East Duarte Road, Duarte, CA 91010, USA
    Leuk Res 31:1749-54. 2007
    b>Systemic mastocytosis (SM) may rarely be associated with lymphoproliferative disorders. In such cases, the relationship between the neoplastic mast cells and the malignant lymphocytes remains unclear...
  32. doi Prognosis in adult indolent systemic mastocytosis: a long-term study of the Spanish Network on Mastocytosis in a series of 145 patients
    Luis Escribano
    Centro de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain
    J Allergy Clin Immunol 124:514-21. 2009
    Indolent systemic mastocytosis is a group of rare diseases for which reliable predictors of progression and outcome are still lacking.
  33. ncbi Bone marrow biopsies for the diagnosis of systemic mastocytosis: is one biopsy sufficient?
    Joseph H Butterfield
    Division of Allergic Diseases and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Am J Clin Pathol 121:264-7. 2004
    ..Bilateral biopsies might be useful for diagnosing early systemic mastocytosis or detecting minimal bone marrow involvement.
  34. ncbi Interest of interferon alpha in systemic mastocytosis. The French experience and review of the literature
    J Simon
    Service de médecine Interne néphrologie, centre hospitalier intercommunal de Poissy Saint Germain en Laye, 10, rue Champ Gaillard, 78300 Poissy, France
    Pathol Biol (Paris) 52:294-9. 2004
    b>Systemic mastocytosis (SM) are defined by an abnormal growth and accumulation of mast cells in bone marrow and/or other extracutaneous organs. There is currently no cure for this disease...
  35. pmc Systemic mastocytosis with associated clonal haematological non-mast cell lineage diseases: a histopathological challenge
    H P Horny
    Institute of Pathology, University of Lubeck, D 23538 Lubeck, Germany
    J Clin Pathol 57:604-8. 2004
    Although systemic mastocytosis (SM) with an associated clonal haematological non-mast cell lineage disease (SM-AHNMD) is a major subtype of SM, little is known about its frequency among myelogenous neoplasms, and mastocytosis in ..
  36. ncbi Tryptase detection in bone-marrow blood: a new diagnostic tool in systemic mastocytosis
    Julia Proelss
    Department of Dermatology, University of Bonn, Bonn, Germany
    J Am Acad Dermatol 56:453-7. 2007
    ..The diagnosis of systemic mastocytosis is substantially based on the histologic examination of bone-marrow biopsy specimens.
  37. ncbi Juxtamembrane-type c-kit gene mutation found in aggressive systemic mastocytosis induces imatinib-resistant constitutive KIT activation
    Nami Nakagomi
    Department of Surgical Pathology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
    Lab Invest 87:365-71. 2007
    Aggressive systemic mastocytosis (ASM) is a very rare form of mast cell neoplasm that does not benefit from conventional chemotherapy...
  38. ncbi CD25 indicates the neoplastic phenotype of mast cells: a novel immunohistochemical marker for the diagnosis of systemic mastocytosis (SM) in routinely processed bone marrow biopsy specimens
    Karl Sotlar
    Institute of Pathology, University of Tubingen, Tubingen, Germany
    Am J Surg Pathol 28:1319-25. 2004
    The diagnosis of systemic mastocytosis (SM) is based primarily on the histologic and immunohistochemical evaluation of a bone marrow trephine biopsy specimen...
  39. ncbi Management of patients with systemic mastocytosis: review of M. D. Anderson Cancer Center experience
    Bryan Hennessy
    Department of Leukemia, The University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Am J Hematol 77:209-14. 2004
    ..g., the bone marrow (systemic mastocytosis; SM). This study objective is to evaluate the features and outcome of patients referred to M. D...
  40. ncbi Successful treatment of systemic mastocytosis with high-dose interferon-alfa: long-term follow-up of a case
    Joseph H Butterfield
    Division of Allergic Diseases and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Leuk Res 29:131-4. 2005
    Daily treatment of systemic mastocytosis with high-dose interferon-alfa often is not tolerated because of clinical or hematologic side effects...
  41. ncbi The systemic mastocytosis-specific activating cKit mutation D816V can be inhibited by the tyrosine kinase inhibitor AMN107
    N von Bubnoff
    Leukemia 19:1670-1. 2005
  42. ncbi Aggressive systemic mastocytosis after germ cell tumor of the ovary: C-KIT mutation documentation in both disease states
    Jae Wook Lee
    Department of Pediatrics, College of Medicine, The Catholic University of Korea, Inchon, Republic of Korea
    J Pediatr Hematol Oncol 29:412-5. 2007
    We report a case of aggressive systemic mastocytosis in a 3-year-old girl, who had undergone treatment for ovarian germ cell tumor during the previous 8 months...
  43. ncbi CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy
    Animesh Pardanani
    Division of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Blood 102:3093-6. 2003
    ..Screening for the FIP1L1-PDGFRA rearrangement and Asp816Val mutation will advance rational therapy decisions in SMCD...
  44. doi Chemotherapy and dasatinib induce long-term hematologic and molecular remission in systemic mastocytosis with acute myeloid leukemia with KIT D816V
    Celalettin Ustun
    Medical College of Georgia, Department of Medicine, Section of Hematology Oncology, Augusta, GA 30912 3125, USA
    Leuk Res 33:735-41. 2009
    ..and molecular remission achieved with combined treatment with chemotherapy and dasatinib in a patient with systemic mastocytosis (SM) and acute myeloid leukemia (AML) with mutant KIT(D816V) expression...
  45. ncbi Fatal bleeding due to a heparin-like anticoagulant in a 37-year-old woman suffering from systemic mastocytosis
    Christoph Sucker
    Department of Hemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Germany
    Clin Appl Thromb Hemost 14:360-4. 2008
    A 37-year-old female patient with systemic mastocytosis who was admitted with severe unexplained bleeding symptoms is studied...
  46. doi Dasatinib therapy for systemic mastocytosis: four cases
    Duncan Purtill
    Eur J Haematol 80:456-8. 2008
  47. doi Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors
    Ken Hong Lim
    Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA
    Blood 113:5727-36. 2009
    Clinical phenotype in systemic mastocytosis (SM) is markedly variable, which complicates prognostication and decision making regarding the choice and timing of therapy...
  48. ncbi Systemic mastocytosis
    Cem Akin
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Annu Rev Med 55:419-32. 2004
    b>Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor...
  49. doi The stromal composition of mast cell aggregates in systemic mastocytosis
    April Chiu
    Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY 10065, USA
    Mod Pathol 22:857-65. 2009
    b>Systemic mastocytosis is a stem cell disorder characterized histologically by the presence of multifocal compact aggregates of mast cells in at least one extracutaneous organ with or without evidence of skin lesions...
  50. ncbi Systemic mastocytosis: current concepts and treatment advances
    Ayalew Tefferi
    Division of Hematology, Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55902, USA
    Curr Hematol Rep 3:197-202. 2004
    b>Systemic mastocytosis (SM), as opposed to cutaneous-only mastocytosis, implies the presence of neoplastic mast cell infiltration in extracutaneous tissue. Mast cell disease in adults is often systemic and often involves the bone marrow...
  51. ncbi Systemic mastocytosis with associated clonal hematological non-mast-cell lineage disease: analysis of clinicopathologic features and activating c-kit mutations
    Vinod A Pullarkat
    Division of Hematology, University of Southern California Keck School of Medicine, Los Angeles, CA, USA
    Am J Hematol 73:12-7. 2003
    The majority of patients with systemic mastocytosis with associated clonal, hematological non-mast cell lineage disease (SM-AHNMD) have a myeloid stem cell malignancy including myelodysplastic syndromes (MDS), myelodysplastic/..
  52. ncbi Identification of MCL1 as a novel target in neoplastic mast cells in systemic mastocytosis: inhibition of mast cell survival by MCL1 antisense oligonucleotides and synergism with PKC412
    Karl J Aichberger
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Austria
    Blood 109:3031-41. 2007
    ..Therefore, MCL-1 has been suggested as a potential new therapeutic target. Systemic mastocytosis (SM) is a myeloid neoplasm involving mast cells (MCs) and their progenitors...
  53. ncbi Treatment of adult systemic mastocytosis with interferon-alpha: results of a multicentre phase II trial on 20 patients
    Philippe Casassus
    Service d Hematologie Clinique, Hopital Avicenne, Universite Paris Nord, Bobigny, Paris, France
    Br J Haematol 119:1090-7. 2002
    b>Systemic mastocytosis (SM) is characterized by proliferation of mast cells in various organs, which may release a wide variety of mediators, thereby explaining the broad clinical spectrum of disease manifestations...
  54. ncbi Diagnosis and treatment of systemic mastocytosis: state of the art
    Peter Valent
    Department of Internal Medicine I, Division of Haematology, University of Vienna, Austria
    Br J Haematol 122:695-717. 2003
  55. ncbi Vesicular monoamine transporter 2 (VMAT2) expression in hematopoietic cells and in patients with systemic mastocytosis
    Martin Anlauf
    Department of Pathology, University of Kiel, Michaelisstr 11, 24105 Kiel, Germany
    J Histochem Cytochem 54:201-13. 2006
    ..study, we analyzed their expression in inflammatory and hematopoietic cells and in patients suffering from systemic mastocytosis (SM) and chronic myelogenous leukemia (CML)...
  56. doi Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates
    A Tefferi
    Divisions of Hematology and Hematopathology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Leukemia 23:900-4. 2009
    ..DNA sequence analysis to screen for TET2 mutations in bone marrow-derived DNA from 48 patients with systemic mastocytosis (SM), including 42 who met the 2008 WHO (World Health Organization) diagnostic criteria for SM and 6 with ..
  57. ncbi The origin of neoplastic mast cells in systemic mastocytosis with AML1/ETO-positive acute myeloid leukemia
    Sumimasa Nagai
    Department of Hematology and Oncology, Graduate School of Medicine and Faculty of Medicine, The University of Tokyo, Bunkyo ku, Tokyo, Japan
    Exp Hematol 35:1747-52. 2007
    b>Systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (SM-AHNMD) is a distinct entity that was defined by World Health Organization...
  58. ncbi Systemic mastocytosis associated with acute myeloid leukemia: case report and implications for disease pathogenesis
    Luis Escribano
    Unidad de Mastocytosis, Laboratorio K Frank Austen, Hospital Ramon y Cajal, Madrid, Spain
    J Allergy Clin Immunol 114:28-33. 2004
    ....
  59. ncbi FIP1L1/PDGFRA is a molecular marker of chronic eosinophilic leukaemia but not for systemic mastocytosis
    P Valent
    Eur J Clin Invest 37:153-4. 2007
  60. ncbi Neoplastic mast cells in systemic mastocytosis associated with t(8;21) acute myeloid leukemia are derived from the leukemic clone
    Vinod Pullarkat
    Division of Hematology and Hematopoietic Cell Transplantation, Department of Cytogenetics, Division of Pathology, City of Hope Comprehensive Cancer Center, 1500 East Duarte Road, Duarte, CA 91010, United States
    Leuk Res 31:261-5. 2007
    In systemic mastocytosis with associated clonal, hematological non-mast cell lineage disease (SM-AHNMD), mast cell infiltration of the bone marrow coexists with a hematologic neoplasm, usually of myeloid origin...
  61. ncbi Rapamycin inhibits growth and survival of D816V-mutated c-kit mast cells
    Marion Gabillot-Carré
    CNRS UMR 8147, Universite Rene Descartes Paris V, Hopital Necker, 161 rue de Sevres, 75743 Paris Cedex 15, France
    Blood 108:1065-72. 2006
    ..stromal tumors (GISTs), and the kinase domain D816V mutation, which is highly representative of systemic mastocytosis (SM)...
  62. ncbi Effects of AMN107, a novel aminopyrimidine tyrosine kinase inhibitor, on human mast cells bearing wild-type or mutated codon 816 c-kit
    Srdan Verstovsek
    Department of Leukemia, University of Texas M D Anderson Cancer Center, Unit 428, 1515 Holcombe Blvd, Houston, TX 77030, United States
    Leuk Res 30:1365-70. 2006
    Most adults with systemic mastocytosis (SM) carry an activating mutation in the codon 816 of c-kit...
  63. ncbi First case of an AIDS patient with systemic mast cell disease associated with FIP1-positive eosinophilia treated with imatinib mesylate therapy
    Serena Merante
    J Clin Oncol 24:e6-7. 2006
  64. ncbi Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment
    Cem Akin
    Department of Internal Medicine, University of Michigan, 4220 D MSRB 3, Box 0638, 1150 West Medical Center Drive, Ann Arbor, MI 48109 0638, USA
    Curr Allergy Asthma Rep 6:282-8. 2006
    ..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 ..
  65. ncbi Mastocytosis: state of the art
    Hans Peter Horny
    Institute of Pathology, Ansbach, Germany
    Pathobiology 74:121-32. 2007
    ..2) Systemic mastocytosis (SM) which is commonly diagnosed in adults and includes four major subtypes: (i) indolent SM (ISM, the ..
  66. ncbi STI571 (Glivec) affects histamine release and intracellular pH after alkalinisation in HMC-1560, 816
    Kristin Löber
    Departamento de Farmacologia, Facultad de Veterinaria, 27002 Lugo, Spain
    J Cell Biochem 103:865-76. 2008
    ..This work is important since HMC-1(560, 816) cells are reported in 80% of aggressive systemic mastocytosis cases and the understanding of some signalling pathways involved in mast cell response could facilitate ..
  67. pmc Unique effects of KIT D816V in BaF3 cells: induction of cluster formation, histamine synthesis, and early mast cell differentiation antigens
    Matthias Mayerhofer
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria
    J Immunol 180:5466-76. 2008
    ..A prototype of an indolent TK-driven neoplasm is indolent systemic mastocytosis. We found that the D816V-mutated variant of KIT, a TK detectable in most patients with systemic ..
  68. ncbi KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities
    Irina Maric
    Department of Laboratory Medicine, Clinical Center, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
    J Allergy Clin Immunol 120:680-7. 2007
    The broad and overlapping clinical manifestations of D816V KIT-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia (CEL), coupled with the increase in activated eosinophils and ..
  69. ncbi Not all c-kit mutations can be corrected by imatinib
    Jerzy Lasota
    Armed Forces Institute of Pathology, Washington, DC, USA
    Lab Invest 87:317. 2007
  70. ncbi Imatinib mesylate in the treatment of hematologic malignancies
    Pier Paolo Piccaluga
    Institute of Haematology and Medical Oncology L and A Seragnoli, S Orsola Malpighi Hospital, University of Bologna, Molecular Pathology Laboratory, Unit of Hematopathology, Via Massarenti, 9 40138 Bologna, Italy
    Expert Opin Biol Ther 7:1597-611. 2007
    ..acute lymphoid leukemia, hypereosinophilic syndromes, gastrointestinal stromal tumours and more recently, systemic mastocytosis and other myeloprolipherative disease-carrying platelet-derived growth factor receptor abnormalities...
  71. ncbi Synergistic growth-inhibitory effects of two tyrosine kinase inhibitors, dasatinib and PKC412, on neoplastic mast cells expressing the D816V-mutated oncogenic variant of KIT
    Karoline V Gleixner
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria
    Haematologica 92:1451-9. 2007
    In a majority of all patients with systemic mastocytosis (SM) including those with mast cell leukemia (MCL), neoplastic mast cells (MC) display the D816V-mutated variant of KIT...
  72. ncbi Quantitative profiling of codon 816 KIT mutations can aid in the classification of systemic mast cell disease
    W Zhao
    Leukemia 21:1574-6. 2007
  73. doi Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusion
    Idoya Lahortiga
    Human Genome Laboratory, Department of Molecular and Developmental Genetics, VIB, Leuven, Belgium
    Haematologica 93:49-56. 2008
    ..We report an unusual case of a patient presenting with peripheral basophilia and systemic mastocytosis in whom cytogenetic analysis revealed a t(4;5)(q21.1;q31.3).
  74. ncbi [Mastocytosis in childhood]
    Joanna Renke
    Klinika Pediatrii, Gastroenterologii i Onkologii, Dzieciecej Akademii Medycznej w Gdańsku
    Przegl Lek 63:551-3. 2006
    ..It concerns 5,4 caser per 1000 of cured children. The disease has no original treatment. There is a chance that thanks to to popularisation of knowledge of this disease will be more frequently diagnosed and known better...
  75. ncbi [Systemic mastocytosis]
    Marek Niedoszytko
    Klinika Alergologii Katedry Pneumonologii i Alergologii Akademii Medycznej, Gdańsku
    Pneumonol Alergol Pol 73:239-44. 2005
    ..The experience of Gdansk Mastocytosis Centre indicates that mastocytosis is a rare and difficult to diagnose disease...
  76. ncbi EXEL-0862, a novel tyrosine kinase inhibitor, induces apoptosis in vitro and ex vivo in human mast cells expressing the KIT D816V mutation
    Jingxuan Pan
    The University of Texas M D Anderson Cancer Center, Unit 428, 1515 Holcombe Blvd, Houston, TX 77030, USA
    Blood 109:315-22. 2007
    Gain-of-function mutations of the receptor tyrosine kinase KIT play a key role in the pathogenesis of systemic mastocytosis (SM), gastrointestinal stromal tumors (GISTs), and some cases of acute myeloid leukemia (AML)...
  77. ncbi Identification of a c-kit exon 8 internal tandem duplication in a feline mast cell tumor case and its favorable response to the tyrosine kinase inhibitor imatinib mesylate
    Mayu Isotani
    Department of Veterinary Clinical Pathology, Nippon Veterinary and Life Science University, 1 7 1 Kyonan cho, Musashino shi, Tokyo 180 8602, Japan
    Vet Immunol Immunopathol 114:168-72. 2006
    ..In the present report, we analyzed the c-kit nucleotide sequence in the case of a cat that showed systemic mastocytosis and mastocytemia...
  78. ncbi Systemic mastocytosis (SM) associated with chronic eosinophilic leukemia (SM-CEL): detection of FIP1L1/PDGFRalpha, classification by WHO criteria, and response to therapy with imatinib
    Stefan Florian
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Wahringer Gurtel 18 20, A 1090 Vienna, Austria
    Leuk Res 30:1201-5. 2006
    Based on generally accepted criteria and the WHO-classification, a subset of patients with systemic mastocytosis (SM) have (or develop) an associated clonal hematologic non-mast cell lineage disease (SM-AHNMD)...
  79. ncbi Imatinib for systemic mast-cell disease
    A Pardanani
    Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN, USA
    Lancet 362:535-6. 2003
    ..The other two, who did not respond to treatment, were the only patients with the ckit D816V mutation. Our results suggest that imatinib either inhibits the growth-promoting role of wild type ckit, or targets an oncogenic kinase...
  80. ncbi Aggressive systemic mastocytosis mimicking sclerosing cholangitis
    Laura Marbello
    Department of Oncology and Hematology, Niguarda Ca Granda Hospital, Milan, Italy
    Haematologica 89:ECR35. 2004
    ..The bone marrow biopsy and the revision of liver biopsy using antitryptase stain diagnosed systemic mastocytosis. Because of the aggressive course of the disease the patient was treated with an acute myeloid leukaemia ..
  81. ncbi Sensitivity of oncogenic KIT mutants to the kinase inhibitors MLN518 and PD180970
    Amie S Corbin
    Oregon Health and Science University Cancer Institute, Portland, OR 97239, USA
    Blood 104:3754-7. 2004
    ..KIT occur in gastrointestinal stromal tumors (GISTs), some cases of acute myelogenous leukemia (AML), and systemic mastocytosis (SM)...
  82. ncbi FIP1L1-PDGFRA and c-kit D816V mutation-based clonality studies in systemic mast cell disease associated with eosinophilia
    Ayalew Tefferi
    Haematologica 89:871-3. 2004
    ..separation to study the extent of clonal involvement by both myeloid and lymphoid cells in 3 patients with systemic mastocytosis associated with eosinophilia...
  83. ncbi Imatinib mesylate (STI571) for myeloid malignancies other than CML
    Geoffrey W Krystal
    Division of Hematology Oncology, Virginia Commonwealth University and McGuire Veterans Affairs Medical Center 111K, 1201 Broad Rock Boulevard, Richmond, VA 23249, USA
    Leuk Res 28:S53-9. 2004
    ..potential role in the treatment of Philadelphia chromosome negative chronic myelogenous leukemia, systemic mastocytosis with associated hematologic neoplasms, chronic myelomonocytic leukemia, specific subtypes of acute ..
  84. ncbi FIP1L1-PDGFRalpha in hypereosinophilic syndrome and mastocytosis
    Gary Gilliland
    Brigham and Women s Hospital, Boston, MA, USA
    Hematol J 5:S133-7. 2004
  85. ncbi Inefficacy of imatinib-mesylate in sporadic, aggressive systemic mastocytosis
    Pellegrino Musto
    Leuk Res 28:421-2. 2004
  86. ncbi Detection of c-kit point mutation Asp-816 --> Val in microdissected pooled single mast cells and leukemic cells in a patient with systemic mastocytosis and concomitant chronic myelomonocytic leukemia
    Karl Sotlar
    Institute of Pathology, University of Tubingen, Liebermeisterstrasse 8, D 72076, Tubingen, Germany
    Leuk Res 26:979-84. 2002
    The c-kit mutation Asp-816-->Val is detectable not only in neoplastic mast cells (MCs) in patients with systemic mastocytosis (SM) but also in most associated hematologic non-MC lineage disease (AHNMD)...
  87. ncbi Effect of tyrosine kinase inhibitor STI571 on the kinase activity of wild-type and various mutated c-kit receptors found in mast cell neoplasms
    Yael Zermati
    CNRS UMR 8603, Hopital Necker, Universite Rene Descartes, Paris, France
    Oncogene 22:660-4. 2003
    b>Systemic mastocytosis (SM) is a rare disease caused by an abnormal mast cell accumulation in various tissues. Two classes of constitutive activating c-kit mutations are found in SM...
  88. ncbi A novel form of mastocytosis associated with a transmembrane c-kit mutation and response to imatinib
    Cem Akin
    Laboratory of Allergic Diseases, National Instititute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Blood 103:3222-5. 2004
    ....
  89. ncbi Clinical, genetic, and therapeutic insights into systemic mast cell disease
    Ayalew Tefferi
    Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA
    Curr Opin Hematol 11:58-64. 2004
    ..Skin-only disease (cutaneous mastocytosis) is infrequent in adults and systemic mastocytosis may be broadly classified as an indolent or aggressive variant based on the absence or presence of ..
  90. ncbi Evolution of urticaria pigmentosa into indolent systemic mastocytosis: abnormal immunophenotype of mast cells without evidence of c-kit mutation ASP-816-VAL
    F Noack
    Department of Pathology, University of Luebeck, Ratzeburger Allee 160, Luebeck, 23538, Germany
    Leuk Lymphoma 44:313-9. 2003
    ..Recently, it could be shown that systemic mastocytosis (SM) is a clonal disorder often exhibiting mutations of c-kit, a protooncogene encoding the tyrosine ..
  91. ncbi 17-Allylamino-17-demethoxygeldanamycin (17-AAG) is effective in down-regulating mutated, constitutively activated KIT protein in human mast cells
    Gerard Fumo
    National Cancer Institute, Cell and Cancer Biology Branch, 9610 Medical Center Dr, Ste 300, Rockville, MD 20850, USA
    Blood 103:1078-84. 2004
    ..These data provide compelling evidence that 17-AAG may be effective in the treatment of c-kit-related diseases including mastocytosis, GISTs, mast cell leukemia, subtypes of acute myelogenous leukemia, and testicular cancer...
  92. ncbi [Treatment of systemic mastocytosis]
    F Marrache
    Fédération de médecine interne, Maladies Infectieuses et Tropicales, Hopital Avicenne, Universite Paris Nord, 125, route de Stalingrad, 93009 Bobigny, France
    Rev Med Interne 24:594-601. 2003
    b>Systemic mastocytosis is a rare disease, characterized by mast cells proliferation in various organs...
  93. pmc Expression of Bcl-2 and Bcl-xL in cutaneous and bone marrow lesions of mastocytosis
    Karin Hartmann
    Department of Dermatology, University of Cologne, Joseph Stelzmann Strasse 9, 50931 Cologne, Germany
    Am J Pathol 163:819-26. 2003
    ..In contrast to the skin lesions, bone marrow infiltrates of patients with systemic mastocytosis showed only low or absent immunoreactivity for bcl-2, but marked expression of bcl-xL...
  94. ncbi Are gastrointestinal mucosal mast cells increased in patients with systemic mastocytosis?
    Sabine I Siegert
    Institute of Pathology, Ludwig Maximilians University, Munich, Germany
    Am J Clin Pathol 122:560-5. 2004
    ..and duodenum and statistically significantly decreased in antrum biopsy specimens from patients with systemic mastocytosis compared with patients with pure urticaria pigmentosa and with control subjects...
  95. ncbi Clonality and molecular pathogenesis of mastocytosis
    Cem Akin
    University of Michigan, Department of Internal Medicine, Division of Allergy and Immunology, 1150 West Medical Center Drive, Ann Arbor, MI 48109 0638, USA
    Acta Haematol 114:61-9. 2005
    ..First, systemic mastocytosis has been found to be associated with activating codon 816 mutations of the c-kit gene...
  96. ncbi Mast cell proliferative disorders: current view on variants recognized by the World Health Organization
    Peter Valent
    Department of Internal Medicine 1, Division of Hematology and Hemostaseology, University of Vienna, Wahringer Gurtel 18 20, Vienna, Austria
    Hematol Oncol Clin North Am 17:1227-41. 2003
    ..In patients with SM-AHNMD, the SM should be treated as if no AHNMD is present, and the AHNMD should be treated as if no SM had been diagnosed...
  97. ncbi A novel NF-kappaB inhibitor, IMD-0354, suppresses neoplastic proliferation of human mast cells with constitutively activated c-kit receptors
    Akane Tanaka
    Laboratory of Molecular Pathology and Therapeutics, Division of Animal Life Science, Graduate School, Institute of Symbiotic Science and Technology, Tokyo University of Agriculture and Technology, Fuchu, Tokyo 183 8509, Japan
    Blood 105:2324-31. 2005
    ..The observations from the current study suggest a therapeutic potential, in systemic mastocytosis, for compounds that interfere with NF-kappaB signaling.
  98. ncbi Systemic mastocytosis: bone marrow pathology, classification, and current therapies
    A Pardanani
    Divisions of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Acta Haematol 114:41-51. 2005
    ..The BM pathology, consensus classification, and current therapies for MCD are further discussed in this article...
  99. ncbi Delineation of patterns of bone marrow mast cell infiltration in systemic mastocytosis: value of CD25, correlation with subvariants of the disease, and separation from mast cell hyperplasia
    Manuela Krokowski
    Institute of Pathology, University of Lubeck, Lubeck, Germany
    Am J Clin Pathol 124:560-8. 2005
    In most cases, the diagnosis of systemic mastocytosis (SM) is based on histomorphologic evaluation of the bone marrow. We analyzed mast cell (MC) infiltration patterns in 57 cases of SM and 31 cases of mast cell hyperplasia (MCH)...
  100. ncbi The faces of mast cell disease: bone marrow infiltrates in 3 patients with systemic mastocytosis
    Antonio E Martinez
    The Arkadi M Rywlin MD, Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA
    Ann Diagn Pathol 9:81-5. 2005
    ..microscopic heterogeneity, particularly in the bone marrow, the most common site of involvement in systemic mastocytosis (SM)...
  101. ncbi Ph-Chromosome-positive chronic myeloid leukemia with associated bone marrow mastocytosis
    Hermine Agis
    Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Austria
    Leuk Res 29:1227-32. 2005
    The concurrent development of chronic myeloid (CML) or myelomonocytic (CMML) leukemia in patients with systemic mastocytosis (SM) is a well recognized phenomenon...

Research Grants66

  1. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2011
    ..tumors, 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...
  2. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2010
    ..tumors, 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...
  3. Role of Vav and Rac in KIT oncogenesis
    Reuben Kapur; Fiscal Year: 2009
    ..tumors, 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...
  4. Activating PTPN11 and c-kit Mutations in Myeloproliferative Disorder
    Rebecca Chan; Fiscal Year: 2007
    ..the receptor protein tyrosine kinase for stem cell factor (SCF), in juvenile myelomonocytic leukemia and systemic mastocytosis, respectively...
  5. 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...
  6. 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 ..
  7. FTI Biology & Treatment of Myeloproliferative Disorders
    Jason Gotlib; Fiscal Year: 2005
    ..If clinically efficacious, further studies of R115777 in MPDs will be warranted...
  8. Stress Induces Skin Mast Cell Activation & Vasodilation
    Theoharis Theoharides; Fiscal Year: 2005
    ..They will also help investigate the pathophysiology of skin syndromes exacerbated by stress, such as atopic dermatitis or psoriasis, especially since CRH and CRH receptors have been identified in human skin. ..
  9. Brain mast cells and Chronic Fatigue Syndrome
    Theoharis C Theoharides; Fiscal Year: 2010
    ..The proposed research is expected to advance our understanding of how activation of brain mast cells can contribute to inflammation and CFS, as well as to the development of novel and effective treatments. ..
  10. Restraint stress-induced neurogenic bladder inflammation
    Theoharis Theoharides; Fiscal Year: 2006
    ..Our findings may be relevant to the pathophysiology of IC and may suggest new therapeutic approaches. ..
  11. CHARACTERIZATION OF DIFFERENT TYPES OF HUMAN MAST CELLS
    Lawrence Schwartz; Fiscal Year: 2007
    ....
  12. 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. ..
  13. STRESS INDUCED SKIN MAST CELL ACTIVATION & VASODILATION
    Theoharis Theoharides; Fiscal Year: 2007
    ..This is an important and under investigated area of skin pathophysiology that has potential applicability to understanding the pathogenesis of skin diseases and screening compounds that may develop into novel and effective treatments. ..
  14. EVALUATION OF BASOPHIL INVOLVEMENT IN HUMAN DISEASE
    Lawrence Schwartz; Fiscal Year: 2001
    ....
  15. 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. ..
  16. CHARACTERIZATION OF DIFFERENT TYPES OF HUMAN MAST CELLS
    Lawrence Schwartz; Fiscal Year: 1993
    ..pathologic conditions, including rheumatoid arthritis, scleroderma, allergic rhinitis, atopic dermatitis, systemic mastocytosis/urticaria pigmentosa, cystic fibrosis, breast and lung solid tumors and AIDS...