Systemic Mastocytosis

313  relevant articles (26 outcomes, 22 trials/studies) found for this Disease

Mastocytosis, Systemic; Aggressive Systemic Mastocytosis; Indolent Systemic Mastocytosis; Aggressive Systemic Mastocytoses; Indolent Systemic Mastocytoses; Mast-Cell Disease, Systemic; Mast-Cell Diseases, Systemic; Mastocytoses, Aggressive Systemic; Mastocytoses, Indolent Systemic; Mastocytoses, Systemic; Mastocytosis, Aggressive Systemic; Mastocytosis, Indolent Systemic; Systemic Mast Cell Disease; Systemic Mast-Cell Diseases; Systemic Mastocytoses; Systemic Mastocytoses, Aggressive; Systemic Mastocytoses, Indolent; Systemic Mastocytosis, Aggressive; Systemic Mastocytosis, Indolent; Systemic Mast-Cell Disease

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Anaphylaxis (Anaphylactic Shock)
2.	Mastocytosis (Mast-Cell Disease)
3.	Myelodysplastic Syndromes (Myelodysplastic Syndrome)
4.	Hypersensitivity (Allergy)
5.	Hypereosinophilic Syndrome (Loeffler Endocarditis)

Experts

1.	Valent, Peter: 7 articles (04/2007 - 03/2002)
2.	Tefferi, Ayalew: 6 articles (06/2008 - 10/2003)
3.	Akin, Cem: 4 articles (06/2008 - 08/2002)
4.	Pardanani, Animesh: 4 articles (06/2008 - 10/2003)
5.	Sperr, Wolfgang R: 4 articles (09/2006 - 03/2002)
6.	Horny, Hans-Peter: 4 articles (04/2006 - 03/2002)
7.	Metcalfe, Dean D: 3 articles (01/2008 - 08/2002)
8.	Agis, Hermine: 3 articles (04/2007 - 03/2005)
9.	Krauth, Maria-Theresa: 3 articles (04/2007 - 03/2005)
10.	Müllauer, Leonhard: 3 articles (04/2007 - 04/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Systemic Mastocytosis:

1.	imatinib (Gleevec)FDA Link 07/15/2006 - "CONCLUSIONS: Imatinib mesylate was effective in patients with systemic mastocytosis, including those who had the D816V mutation." 07/15/2006 - "Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial." 05/01/2008 - "One patient with systemic mastocytosis and a partial response to therapy had a novel imatinib-sensitive KIT mutation (D816T)" 01/01/2008 - "This work also illustrates the use of imatinib for the treatment of specific cases of systemic mastocytosis." 10/01/2007 - "Systemic Mastocytosis: documented pathologic response to imatinib." Order ALL the reference details at left...
2.	Interferon-alpha (Interferon Alfa)FDA Link 01/01/1995 - "Efficacy of interferon alpha in a case of systemic mastocytosis]" 12/01/2002 - "Treatment of adult systemic mastocytosis with interferon-alpha: results of a multicentre phase II trial on 20 patients." 02/01/2005 - "Daily treatment of systemic mastocytosis with high-dose interferon-alfa often is not tolerated because of clinical or hematologic side effects" 02/01/2005 - "Successful treatment of systemic mastocytosis with high-dose interferon-alfa: long-term follow-up of a case." 07/01/2004 - "A 53-year old patient with beginning "smoldering systemic mastocytosis" failed to respond to high-dose systemic glucocorticosteroids and interferon-alpha" Order ALL the reference details at left...
3.	Cromolyn Sodium (Cromoglicic Acid)FDA LinkGeneric 05/01/1990 - "A multicenter, double-blind, placebo-controlled trial of the efficacy of oral cromolyn sodium (200 mg orally four times per day) was conducted in 11 patients with systemic mastocytosis who had been maintained with the drug on an individualized compassionate-need basis" 08/30/1979 - "A double-blind crossover study of the efficacy of disodium cromoglycate given by mouth to control the cutaneous, gastrointestinal and central-nervous-system manifestations of systemic mastocytosis was carried out in five patients for periods of eight to 32 months" 05/01/1990 - "Cromolyn sodium in the management of systemic mastocytosis." 01/01/1985 - "This is the first reported case of systemic mastocytosis involving only bone which responded to treatment with oral disodium cromoglycate." 01/01/1985 - "Disodium cromoglycate in the treatment of systemic mastocytosis involving only bone." Order ALL the reference details at left...
4.	VenomsIBA 09/01/1994 - "Rush Hymenoptera venom immunotherapy: successful treatment in a patient with systemic mast cell disease." 08/01/2004 - "RECENT FINDINGS: Systemic mastocytosis is overrepresented in the Hymenoptera-allergic population and is associated with an increased risk of more-severe reactions following field stings or sting challenges, lack of sensitization to venoms by skin test or radioallergosorbent test, increased side effects to venom immunotherapy, reduced efficacy of venom immunotherapy and treatment failures" 02/01/2008 - "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" 02/01/2008 - "Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis." 06/01/2007 - "In part 2 of this series, we will discuss the use of venom immunotherapy, the diagnosis of systemic mastocytosis that initially presents as anaphylaxis, and the efficacy of immunotherapy in patients with mastocytosis." Order ALL the reference details at left...
5.	Tryptases (Tryptase)IBA 03/01/2007 - "CONCLUSION: Our results indicate that the measurement of tryptase in bone-marrow blood is a new, sensitive marker of the mast cell burden in bone marrow of patients with systemic mastocytosis." 03/01/2007 - "RESULTS: We could detect for the first time significantly elevated tryptase levels in bone-marrow blood of patients with systemic mastocytosis" 03/01/2007 - "Bone-marrow biopsy specimens of patients with systemic mastocytosis (n = 7) and control subjects (n = 7) were investigated histologically and bone-marrow blood of these individuals was analyzed on the tryptase levels" 03/01/2007 - "Tryptase detection in bone-marrow blood: a new diagnostic tool in systemic mastocytosis." 01/01/2007 - "RESULTS: In 3 patients, the baseline serum tryptase level was elevated (26, 36, and 67 ng/ml, respectively), and investigation of their bone marrow revealed systemic mastocytosis (SM)" Order ALL the reference details at left...
6.	dasatinib (BMS 354825)FDA Link 05/01/2008 - "Dasatinib therapy for systemic mastocytosis: four cases." 07/01/2006 - "Significantly, dasatinib selectively kills primary neoplastic bone marrow mast cells from patients with systemic mastocytosis while sparing other hematopoietic cells" 06/15/2008 - "Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis." 07/01/2006 - "Dasatinib (BMS-354825) inhibits KITD816V, an imatinib-resistant activating mutation that triggers neoplastic growth in most patients with systemic mastocytosis." 08/01/2007 - "Novel tyrosine kinase inhibitors such as imatinib, nilotinib and dasatinib, have been found to be effective in the management of various subtypes of systemic mastocytosis and gastrointestinal stromal tumors" Order ALL the reference details at left...
7.	Codon (Codons)IBA 08/01/2008 - "A case of systemic mastocytosis carrying the characteristic mutation at codon 816 (D816V) in the KIT gene of mast cells, with two concurrent accompanying clonal haematopoietic non-mast cell-lineage disorders, chronic myeloproliferative disease, unclassifiable and precursor B lymphoblastic leukaemia is documented" 07/01/2007 - "Quantitative profiling of codon 816 KIT mutations can aid in the classification of systemic mast cell disease." 11/01/2006 - "Most adults with systemic mastocytosis (SM) carry an activating mutation in the codon 816 of c-kit" 09/01/2006 - "Detection of a codon 816 c-kit mutation is included as a minor diagnostic criterion in the World Health Organization's diagnostic criteria for systemic mastocytosis" 06/01/2005 - "In systemic mastocytosis (SM), neoplastic cells are driven by the RTK KIT, which is mutated at codon 816 in most patients" Order ALL the reference details at left...
8.	Protein-Tyrosine Kinases (Tyrosine Kinase)IBA 10/01/2007 - "Detection of phospho-STAT5 in mast cells: a reliable phenotypic marker of systemic mast cell disease that reflects constitutive tyrosine kinase activation." 07/01/2004 - "Finally, it would be possible to design, in the very next future, new tyrosine kinase inhibitors targeting specifically the mutant forms of c-Kit found in patients suffering from systemic mastocytosis." 09/01/2005 - "The systemic mastocytosis-specific activating cKit mutation D816V can be inhibited by the tyrosine kinase inhibitor AMN107." 02/01/2003 - "Recently, it could be shown that systemic mastocytosis (SM) is a clonal disorder often exhibiting mutations of c-kit, a protooncogene encoding the tyrosine kinase receptor for stem cell factor (SCF)" 01/01/2000 - "The authors have employed immunohistochemistry for the C-KIT encoded tyrosine kinase receptor protein, CD117, for detection of mast cells on paraffin sections of 89 bone marrow specimens including systemic mast cell disease and other disorders" Order ALL the reference details at left...
9.	Platelet-Derived Growth Factor Receptors (Platelet-Derived Growth Factor Receptor)IBA 01/01/2007 - "Imatinib mesylate responsiveness in aggressive systemic mastocytosis: novel association with a platelet derived growth factor receptor beta mutation." 06/15/2003 - "Although bone marrow biopsies in these patients showed increased numbers of CD25+ mast cells and atypical spindle-shaped mast cells, patients with HES and elevated serum tryptase could be distinguished from patients with systemic mastocytosis and eosinophilia by their clinical manifestations, the absence of mast cell aggregates, the lack of a somatic KIT mutation, and the presence of the recently described fusion of the Fip1-like 1 (FIP1L1) gene to the platelet-derived growth factor receptor alpha gene (PDGFRA)" 10/01/2007 - "Since then, imatinib became the first molecular-targeted agent approved for the treatment of human cancer and was later on demonstrated to be effective in other malignancies, such as Philadelphia positive acute lymphoid leukemia, hypereosinophilic syndromes, gastrointestinal stromal tumours and more recently, systemic mastocytosis and other myeloprolipherative disease-carrying platelet-derived growth factor receptor abnormalities" 04/01/2006 - "The current review focuses on the diagnosis and treatment of both molecularly defined and clinicopathologically assigned categories of atypical MPD: chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, chronic neutrophilic leukemia, chronic basophilic leukemia, chronic eosinophilic leukemia, idiopathic eosinophilia including hypereosinophilic syndrome, systemic mastocytosis, unclassified MPD, and eosinophilic/mast cell disorders associated with mutations of platelet-derived growth factor receptors alpha (PDGFRA) and beta (PDGFRB), FGFR1, and KIT." 09/15/2008 - "Expression of the fusion gene FIP1-like 1/platelet-derived growth factor receptor alpha (FIP1L1/PDGFRalpha, F/P) and dysregulated c-kit tyrosine kinase activity are associated with systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL)/hypereosinophilic syndrome (HES)" Order ALL the reference details at left...
10.	CladribineFDA LinkGeneric 12/15/2003 - "Single-agent cladribine is an effective and relatively safe treatment for severe systemic mastocytosis" 02/01/2004 - "Treatment of systemic mast cell disease with 2-chlorodeoxyadenosine." 02/01/2004 - "Cladribine activity in systemic mast cell disease: application of biochemistry in the modern era of molecular biology." 12/15/2003 - "Cladribine therapy for systemic mastocytosis." 01/25/2001 - "Treatment of systemic mast-cell disease with cladribine." Order ALL the reference details at left...

Therapies and Procedures

1.	Immunotherapy 01/01/1999 - "Patients with systemic mastocytosis are at risk for significant mast cell mediator release during immunotherapy, conventional or alternative." 01/01/2008 - "Allergen specific immunotherapy is safe and effective in patients with systemic mastocytosis and Hymenoptera allergy." 07/01/2007 - "We further discuss the diagnosis of systemic mastocytosis, initially presenting as anaphylaxis, and the efficacy of immunotherapy in patients with mastocytosis." 09/01/1994 - "Rush Hymenoptera venom immunotherapy: successful treatment in a patient with systemic mast cell disease." 08/01/2004 - "RECENT FINDINGS: Systemic mastocytosis is overrepresented in the Hymenoptera-allergic population and is associated with an increased risk of more-severe reactions following field stings or sting challenges, lack of sensitization to venoms by skin test or radioallergosorbent test, increased side effects to venom immunotherapy, reduced efficacy of venom immunotherapy and treatment failures" Order ALL the reference details at left...
2.	Splenectomy 12/01/1979 - "Systemic mastocytosis in a cat: remission after splenectomy." 06/01/1997 - "Systemic mastocytosis was confirmed by splenectomy, liver biopsy and finally autopsy" 01/01/1990 - "The records of 26 patients with systemic mast cell disease (SMCD) treated during the past decade at the National Institutes of Health were reviewed to determine the role of splenectomy in the management of SMCD" 01/01/1990 - "Splenectomy in the management of systemic mast cell disease." 04/01/2008 - "METHODS: We describe a case of PVT in a woman who underwent laparoscopic splenectomy for symptomatic splenomegaly secondary to systemic mastocytosis" Order ALL the reference details at left...
3.	Drug Therapy (Chemotherapy) 07/01/1997 - "We report a rare case of systemic mastocytosis causing intrahepatic cholestasis that resolved with remission of AML following chemotherapy." 04/01/2007 - "Aggressive systemic mastocytosis (ASM) is a very rare form of mast cell neoplasm that does not benefit from conventional chemotherapy" 12/01/1998 - "A 12-year-old girl with a mixed malignant germ cell tumor of the ovary, treated by surgery and chemotherapy, developed systemic mast cell disease (SMCD) approximately 3 months after chemotherapy" 07/01/2004 - "Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy" Order ALL the reference details at left...
4.	Combination Drug Therapy (Combination Chemotherapy) 09/15/1993 - "Fourteen patients were treated by initial high-dose cisplatin combination chemotherapy and 8 (57%) achieved complete remission (2 died of systemic mastocytosis development)" 02/01/1999 - "We report here, to our knowledge, the first case of Hodgkin's and Castleman's disease occurring in a patient with co-existent systemic mastocytosis, which remained unchanged after combination chemotherapy for Hodgkin's disease." Order ALL the reference details at left...
5.	Stem Cell Transplantation 08/01/2008 - "The chronic myeloproliferative disease, unclassifiable and the precursor B lymphoblastic leukaemia were cured by syngenous stem cell transplantation, but the systemic mastocytosis persisted for more than 10 years" Order ALL the reference details at left...