The Mastocytosis Society Survey On Mast Cell Disorders: Patient Experiences and Perceptions

Jennings, Susan V.; Russell, Nancy; Jennings, Blair; Slee, Valerie M.; Sterling, Lisa; Castells, Mariana; Valent, Peter; Akin, Cem

doi:10.1016/j.jaci.2012.12.1087

Cited by 43 publications

(79 citation statements)

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“…Depression and asthenia are potential psychiatric manifestations of mast cell activation and can have a negative infl uence on the wellbeing of patients with systemic mastocytosis. 1,2,22,23 Thus, the improvement in endpoints, such as the Fatigue Impact Scale and Hamilton Rating Scale for Depression (non-signifi cant), and their associated 4R75% and 3R75% composite endpoints is indicative that masitinib can positively aff ect neuropsychiatric manifestations of systemic mastocytosis. These effi cacy data confi rm observations from related phase 2 studies (appendix p 20).…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Although life expectancy is similar to that of the general population in the relatively indolent variants of mastocytosis-cutaneous mastocytosis and indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosisabout a third of patients will experience severe symptoms of mast cell mediator release. A greatly increased occurrence and severity of such symptoms is reported in systemic mastocytosis when compared with cutaneous mastocytosis.…”

Section: Introductionmentioning

confidence: 99%

“…A greatly increased occurrence and severity of such symptoms is reported in systemic mastocytosis when compared with cutaneous mastocytosis. 1,2 Treatment decisions are based on the presence of bone marrow mast cell infi ltration and severity of symptoms, with the main objective being a sustained improvement of symptoms-ie, inhibition of mast cell mediator release.…”

Section: Introductionmentioning

confidence: 99%

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Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study

Lortholary¹,

Chandesris²,

Livideanu³

et al. 2017

Preprint

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sitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebocontrolled, phase 3 study. The Lancet, Elsevier, 2017, 389 (10069) SummaryBackground Indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with reduced quality of life. Masitinib inhibits KIT and LYN kinases that are involved in indolent systemic mastocytosis pathogenesis. We aimed to assess safety and effi cacy of masitinib versus placebo in severely symptomatic patients who were unresponsive to optimal symptomatic treatments.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study

Lortholary¹,

Chandesris²,

Livideanu³

et al. 2017

Preprint

View full text Add to dashboard Cite

show abstract

“…It should be noted, however, that the serum levels in the control subjects in these studies were unusually high (e.g., 5.46 6 1.38 pg/ml; Gur et al, 2002) compared with The increased IL-6 levels may derive from MCs because acute-restraint physiologic stress of mice led to increased serum IL-6, which was entirely MC-dependent (Geiss et al, 2011). Moreover, increased serum IL-6 was associated with bone pain in patients with mastocytosis (Theoharides et al, 2015f), who often present with comorbid FMS (Jennings et al, 2014;Theoharides et al, 2015f). It is important to note that patients with mastocytosis experience diffuse musculoskeletal pain (Delsignore et al, 1996) and are often diagnosed with FMS (Theoharides et al, 2015c).…”

Section: Discussionmentioning

confidence: 99%

Neuropeptides CRH, SP, HK-1, and Inflammatory Cytokines IL-6 and TNF Are Increased in Serum of Patients with Fibromyalgia Syndrome, Implicating Mast Cells

Tsilioni

Russell

Stewart

et al. 2016

Journal of Pharmacology and Experimental Therapeutics

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Fibromyalgia syndrome (FMS) is a chronic, idiopathic condition of widespread musculoskeletal pain affecting more women than men. Even though clinical studies have provided evidence of altered central pain pathways, the lack of definitive pathogenesis or reliable objective markers has hampered development of effective treatments. Here we report that the neuropeptides corticotropin-releasing hormone (CRH), substance P (SP), and SP-structurally-related hemokinin-1 (HK-1) were significantly (P 5 0.026, P , 0.0001, and P 5 0.002, respectively) elevated (0.82 6 0.57 ng/ml, 0.39 6 0.18 ng/ml, and 7.98 6 3.12 ng/ml, respectively) in the serum of patients with FMS compared with healthy controls (0.49 6 0.26 ng/ml, 0.12 6 0.1 ng/ml, and 5.71 6 1.08 ng/ml, respectively). Moreover, SP and HK-1 levels were positively correlated (Pearson r 5 0.45, P 5 0.002) in FMS. The serum concentrations of the inflammatory cytokines interleukin (IL)-6 and tumor necrosis factor (TNF) were also significantly (P 5 0.029 and P 5 0.006, respectively) higher (2.97 6 2.35 pg/ml and 0.92 6 0.31 pg/ml, respectively) in the FMS group compared with healthy subjects (1.79 6 0.62 pg/ml and 0.69 6 0.16 pg/ml, respectively). In contrast, serum IL-31 and IL-33 levels were significantly lower (P 5 0.0001 and P 5 0.044, respectively) in the FMS patients (849.5 6 1005 pg/ml and 923.2 6 1284 pg/ml, respectively) in comparison with healthy controls (1281 6 806.4 pg/ml and 3149 6 4073 pg/ml, respectively). FMS serum levels of neurotensin were not different from controls. We had previously shown that CRH and SP stimulate IL-6 and TNF release from mast cells (MCs). Our current results indicate that neuropeptides could stimulate MCs to secrete inflammatory cytokines that contribute importantly to the symptoms of FMS. Treatment directed at preventing the secretion or antagonizing these elevated neuroimmune markers, both centrally and peripherally, may prove to be useful in the management of FMS.

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“…However, the risks of pursuing this study include cost and subjecting patients to an often invasive and uncomfortable procedure. Even when performed appropriately, mastocytosis may be missed [33] and bone marrow biopsy may be performed incorrectly [34]. Alvarez-Twose et al has proposed using the Spanish Network on Mastocytosis (REMA) score to screen for a clonal mast cell disorder after demonstrating that it has better specificity but similar sensitivity to tryptase levels, with a score of two or more indicating a high probability for clonal mast cell disorder [28, 35•].…”

Section: Diagnosis Of Idiopathic Anaphylaxis-the Role Of Bone Marrow mentioning

confidence: 99%

Idiopathic Anaphylaxis—A Diagnostic and Therapeutic Dilemma

Kim

Khan

2015

Curr Treat Options Allergy

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The Mastocytosis Society Survey On Mast Cell Disorders: Patient Experiences and Perceptions

Cited by 43 publications

References 0 publications

Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study

Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study

Neuropeptides CRH, SP, HK-1, and Inflammatory Cytokines IL-6 and TNF Are Increased in Serum of Patients with Fibromyalgia Syndrome, Implicating Mast Cells

Idiopathic Anaphylaxis—A Diagnostic and Therapeutic Dilemma

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