|1.||Anaphylaxis (Anaphylactic Shock)
|2.||Mastocytosis (Mast-Cell Disease)
|5.||Myelodysplastic Syndromes (Myelodysplastic Syndrome)
|1.||Valent, Peter: 32 articles (12/2015 - 01/2002)|
|2.||Tefferi, Ayalew: 19 articles (03/2010 - 11/2002)|
|3.||Horny, Hans-Peter: 18 articles (12/2015 - 01/2002)|
|4.||Akin, Cem: 18 articles (10/2014 - 07/2002)|
|5.||Sperr, Wolfgang R: 16 articles (10/2014 - 01/2002)|
|6.||Valent, P: 15 articles (05/2015 - 08/2000)|
|7.||Pardanani, Animesh: 13 articles (03/2010 - 11/2002)|
|8.||Sotlar, Karl: 12 articles (12/2015 - 01/2005)|
|9.||Metcalfe, Dean D: 11 articles (12/2015 - 07/2002)|
|10.||Escribano, Luis: 10 articles (11/2015 - 01/2003)|
|1.||imatinib (Gleevec)FDA Link
01/01/2009 - "Patients with CEL and HES responded to imatinib and remained in complete haematological, clinical and molecular (for carriers of FIP1L1-PDGFRA rearrangement) remission for a median of 28.2 months (range: 11-54), whilst the patient with systemic mastocytosis did not respond. "
07/15/2006 - "Imatinib mesylate was effective in patients with systemic mastocytosis, including those who had the D816V mutation."
11/01/2009 - "Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis."
07/15/2006 - "Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial."
12/01/2015 - "In systemic mastocytosis (SM), KIT D816V is identified in ∼90 % of patients, but demonstrates imatinib resistance. "
05/01/2011 - "Hymenoptera venom is one of the best known mediators-release trigger in patients with systemic mastocytosis (SM). "
09/01/1994 - "Rush Hymenoptera venom immunotherapy: successful treatment in a patient with systemic mast cell disease."
05/01/2011 - "The aim of the study was to analyze differences in gene expression between patients with indolent systemic mastocytosis (ISM) and a history of insect venom anaphylaxis (IVA) compared to those patients without a history of anaphylaxis, and to determine the predictive use of gene expression profiling. "
07/01/2013 - "In patients with indolent systemic mastocytosis with a history of Hymenoptera venom exposure after age 15 years or greater (n = 152), MIMA and age at the most recent Hymenoptera sting were independent predictors for HVAn (odds ratios of 0.723 [P = .001] and 1.062 [P < .001], respectively). "
05/01/2011 - "Gene expression analysis predicts insect venom anaphylaxis in indolent systemic mastocytosis."
|3.||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. "
|4.||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."
|5.||Protein-Tyrosine Kinases (Tyrosine Kinase)IBA
05/01/2014 - "D816V KIT is found in approximately 80% of the patients with systemic mastocytosis, and is resistant to the first and second generations of tyrosine kinase inhibitors (TKI). "
01/01/2014 - "Subsequently, the FIP1L1-PDGFRα (F/P) fusion tyrosine kinase was identified in patients with HES and found to be the most common clonal defect in CEL and the second most frequent mutation in systemic mastocytosis (SM). "
07/01/2012 - "Most patients with systemic mastocytosis bear mutations in the tyrosine kinase receptor gene c-Kit. "
09/01/2011 - "Systemic mastocytosis (SM) is a heterogeneous disease, vast majority of these patients have a gain of function mutation in the gene encoding the tyrosine kinase KIT (KIT(D816V)). "
09/01/2011 - "Tyrosine kinase inhibitors in the treatment of systemic mastocytosis."
|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."
05/01/2009 - "Here, we present the first case report of long-term hematologic 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. "
05/01/2009 - "Chemotherapy and dasatinib induce long-term hematologic and molecular remission in systemic mastocytosis with acute myeloid leukemia with KIT D816V."
|7.||omalizumab (Xolair)FDA Link
07/01/2010 - "Omalizumab is effective in treating systemic mastocytosis in a nonatopic patient."
01/01/2015 - "This observation provides additional insight into the effects of omalizumab on systemic mastocytosis. "
01/01/2015 - "Cutaneous and gastrointestinal symptoms in two patients with systemic mastocytosis successfully treated with omalizumab."
01/01/2011 - "We describe an unexpected discontinuation of omalizumab following successful initiation of VIT in a patient with systemic mastocytosis, with subsequent lack of tolerability of VIT. "
05/01/2014 - "Omalizumab induced remission of idiopathic anaphylaxis in a patient suffering from indolent systemic mastocytosis."
12/15/2003 - "Single-agent cladribine is an effective and relatively safe treatment for severe systemic mastocytosis. "
05/01/2011 - "Cladribine therapy was efficient in the patient' with systemic mastocytosis but the response was transient, so there is the need to search for new therapeutic options and more effective strategies in the treatment of patients with aggressive mast cell disorders."
05/01/2011 - "Response to cladribine in patient with 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."
12/01/2015 - "Tryptase levels and symptoms decreased over time in most patients, and tryptase levels correlated with bone marrow mast cell burden in patients with systemic mastocytosis (P < .0001). "
03/01/2015 - "Currently, measurement of serum tryptase level is the most commonly used test to estimate the need for bone marrow biopsy in patients suspected to have indolent systemic mastocytosis (ISM). "
03/01/2015 - "Having found elevated tryptase levels, these were followed by a bone marrow biopsy to rule out systemic mastocytosis. "
02/01/2014 - "Indolent systemic mastocytosis (ISM) without skin lesions (ISMs(-)) shows a higher prevalence in males, lower serum baseline tryptase levels, and KIT mutation more frequently restricted to bone marrow (BM) mast cells (MCs) than ISM with skin lesions (ISMs(+)). "
10/01/2013 - "Lack of evidence for intrathecal tryptase synthesis in patients with systemic mastocytosis."
|10.||4- methyl- N- (3- (4- methylimidazol- 1- yl)- 5- (trifluoromethyl)phenyl)- 3- ((4- pyridin- 3- ylpyrimidin- 2- yl)amino)benzamide (nilotinib)FDA Link
11/01/2015 - "Nilotinib in patients with systemic mastocytosis: analysis of the phase 2, open-label, single-arm nilotinib registration study."
09/01/2005 - "The systemic mastocytosis-specific activating cKit mutation D816V can be inhibited by the tyrosine kinase inhibitor AMN107."
08/01/2010 - "OM cells of five patients with CML and one patient with systemic mastocytosis treated with imatinib or nilotinib and of eight patients with RCC or HCC treated with sorafenib or sunitinib showed centrosome defects in a mean of 15%. "
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. "
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 - "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. "
|2.||Drug Therapy (Chemotherapy)
11/01/2012 - "In two cases of CBF AML associated with systemic mastocytosis (SM), the persistence of mast cells and the detection of a high ratio of fusion transcript, in bone marrow, during the courses of chemotherapy, led us to determine whether the mast cell component of the disease carried the same molecular alterations as leukaemic blasts. "
03/01/2010 - "The current review provides an update on prognosis and treatment of systemic mastocytosis, including investigational drug therapy. "
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. "
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. "
04/01/2008 - "We describe a case of PVT in a woman who underwent laparoscopic splenectomy for symptomatic splenomegaly secondary to systemic mastocytosis. "
04/01/2008 - "Portal vein thrombosis after laparoscopic splenectomy for systemic mastocytosis: a case report and review of the literature."
08/01/1987 - "Histamine levels and cardiovascular responses during splenectomy and splenorenal shunt formation in a patient with systemic mastocytosis."
|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."
|5.||Stem Cell Transplantation