Cytogenetic abnormalities in systemic mastocytosis: WHO subcategory‐specific incidence and prognostic impact among 348 informative cases
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DOI: 10.1002/ajh.25265
Abstract: The World Health Organization (WHO) system lists five morphological categories of systemic mastocytosis (SM): indolent (ISM), smoldering, SM with an associated hematological neoplasm (SM‐AHN), aggressive (ASM) and mast cell leukemia (MCL). Recent studies have highlighted… read more here.
Keywords: incidence; cytogenetic abnormalities; systemic mastocytosis; abnormal karyotype ... See more keywords
Midostaurin therapy for advanced systemic mastocytosis: Mayo Clinic experience in 33 consecutive cases
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DOI: 10.1002/ajh.26498
Abstract: We retrospectively examined our experience with midostaurin therapy in 33 consecutive patients (median age 68 years; 58% females) with advanced systemic mastocytosis (adv‐SM): aggressive SM (ASM; n = 17), SM associated with another hematologic neoplasm (SM‐AHN;… read more here.
Keywords: therapy; systemic mastocytosis; midostaurin therapy; advanced systemic ... See more keywords
Altered innate immune profile in blood of systemic mastocytosis patients
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DOI: 10.1002/clt2.12167
Abstract: Abstract Background Mast cells (MC) from systemic mastocytosis (SM) patients release MC mediators that lead to an altered microenvironment with potential consequences on innate immune cells, such as monocytes and dendritic cells (DC). Here we… read more here.
Keywords: systemic mastocytosis; innate immune; blood; blood monocytes ... See more keywords
Patient‐reported outcomes among patients with systemic mastocytosis in routine clinical practice: Results of the TouchStone SM Patient Survey
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DOI: 10.1002/cncr.34420
Abstract: Systemic mastocytosis (SM) is a rare clonal neoplasm driven by KIT D816V and other mutations. Data were collected from the patient perspective on disease burden and included an SM‐specific symptom assessment tool. read more here.
Keywords: reported outcomes; mastocytosis; patient reported; systemic mastocytosis ... See more keywords
Therapeutic management and outcome of patients with advanced systemic mastocytosis treated with midostaurin: A comprehensive real‐life study in the French national healthcare database
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DOI: 10.1002/hon.3062
Abstract: Advanced Systemic Mastocytosis (Adv‐SM) is rare and has a poor prognosis. Midostaurin (Rydapt®) is one of the few treatments for Adv‐SM in Europe. The study aims were to describe the characteristics of patients treated with… read more here.
Keywords: systemic mastocytosis; advanced systemic; treatment; treated midostaurin ... See more keywords
Multiple vertebral fractures as the first manifestation of systemic mastocytosis
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DOI: 10.1007/s00198-019-04897-9
Abstract: Systemic mastocytosis is a clonal disease of the mast cell progenitors of the bone marrow. The clinical picture varies from asymptomatic (indolent) to highly aggressive (mast cell leukemia). Up to one-third of patients with SM… read more here.
Keywords: multiple vertebral; fractures first; systemic mastocytosis; first manifestation ... See more keywords
Protease profile of normal and neoplastic mast cells in the human bone marrow with special emphasis on systemic mastocytosis
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DOI: 10.1007/s00418-021-01964-3
Abstract: Mast cells (MC) are immune cells that produce a variety of mediators, such as proteases, that are important in the body’s immune responses. MC proteases have pronounced multifunctionality and in many respects determine the biological… read more here.
Keywords: mast cells; bone marrow; mastocytosis; systemic mastocytosis ... See more keywords
A rare manifestation of indolent systemic mastocytosis and its management during the coronavirus disease 2019 pandemic; educational lessons from Syria
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DOI: 10.1016/j.amsu.2021.01.062
Abstract: The diagnosis of indolent systemic mastocytosis can be quite a challenge due to its wide spectrum of clinical manifestations. We are reporting a case of misdiagnosed indolent systemic mastocytosis in a 41-year-old male that has… read more here.
Keywords: indolent systemic; disease 2019; coronavirus disease; systemic mastocytosis ... See more keywords
Systemic mastocytosis associates with cardiovascular events despite lower plasma lipid levels.
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DOI: 10.1016/j.atherosclerosis.2017.11.030
Abstract: BACKGROUND AND AIMS Mast cells have been implicated in the development and progression of atherosclerosis in animal models and human autopsy studies. However, it is unknown whether long-term exposure to excess of mast cells is… read more here.
Keywords: prevalence; systemic mastocytosis; cvd; despite lower ... See more keywords
Tyrosine Kinase Inhibitors in the Treatment of Eosinophilic Neoplasms and Systemic Mastocytosis.
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DOI: 10.1016/j.hoc.2017.04.009
Abstract: The World Health Organization's semimolecular classification of eosinophilias emphasizes neoplasms driven by fusion tyrosine kinases. More than 80% of patients with systemic mastocytosis carry the KIT D816V mutation, the primary driver of disease pathogenesis. Genetic… read more here.
Keywords: kinase inhibitors; eosinophilic neoplasms; systemic mastocytosis; tyrosine kinase ... See more keywords
SETD2 and histone H3 lysine 36 methylation deficiency in advanced systemic mastocytosis
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DOI: 10.1038/leu.2017.183
Abstract: The molecular basis of advanced systemic mastocytosis (SM) is not fully understood and despite novel therapies the prognosis remains dismal. Exome sequencing of an index-patient with mast cell leukemia (MCL) uncovered biallelic loss-of-function mutations in… read more here.
Keywords: setd2 histone; setd2; systemic mastocytosis; advanced systemic ... See more keywords