ライフサイエンスコーパス: mastocytosis

1 e MQLQ and MSAF with independent measures of mastocytosis.

2 atients with cutaneous and indolent systemic mastocytosis.

3 of life (HRQoL) impairment in patients with mastocytosis.

4 L-6R were similarly reduced in patients with mastocytosis.

5 ific quality-of-life (QoL) for patients with mastocytosis.

6 ), nodular (6%), and diffuse cutaneous (12%) mastocytosis.

7 gnificant determinant of HRQoL impairment in mastocytosis.

8 correlate well with bone marrow findings of mastocytosis.

9 articularly in patients with childhood-onset mastocytosis.

10 n associated with several diseases including mastocytosis.

11 ersistent disease resembling adulthood-onset mastocytosis.

12 ed cold urticaria, mosquito bite allergy and mastocytosis.

13 cacy of cladribine in 68 adult patients with mastocytosis.

14 assessed in those patients with ascertained mastocytosis.

15 eases associated with KIT mutations, such as mastocytosis.

16 a clinically diverse cohort of patients with mastocytosis.

17 mug/l and anaphylaxis may have an underlying mastocytosis.

18 nfiltrating the bone marrow of patients with mastocytosis.

19 ow and peripheral blood of 105 patients with mastocytosis.

20 nostic algorithm for patients with suspected mastocytosis.

21 s and pathogenic ILC2-mast cell crosstalk in mastocytosis.

22 e myeloid leukemia, testicular seminomas and mastocytosis.

23 gastrointestinal symptoms for patients with mastocytosis.

24 to improve prognostication for patients with mastocytosis.

25 rointestinal manifestations in patients with mastocytosis.

26 emic mastocytosis accounting for < 1% of all mastocytosis.

27 T levels might also indicate the presence of mastocytosis.

28 and TrkC on intestinal MCs of patients with mastocytosis.

29 h increasing mast cell load in patients with mastocytosis.

30 allergies, other inflammatory reactions, and mastocytosis.

31 ource of IL-9, they might support esophageal mastocytosis.

32 ast cell load and HVAn risk in patients with mastocytosis.

33 for severe MC activation events in pediatric mastocytosis.

34 drug resistance in neoplastic MC in advanced mastocytosis.

35 rker to diagnose and predict severe forms of mastocytosis.

36 nd in the majority of patients with systemic mastocytosis.

37 f conventional drugs for aggressive systemic mastocytosis.

38 ciated with the myeloproliferative disorder, mastocytosis.

39 the suppression of mast cell disease such as mastocytosis.

40 systemic mastocytosis variant of smoldering mastocytosis.

41 al time points in two patients with systemic mastocytosis.

42 marrow mast cells in patients with systemic mastocytosis.

43 g new treatment options in advanced systemic mastocytosis.

44 WHO, and the European Competence Network on Mastocytosis.

45 symptomatic indolent or smouldering systemic mastocytosis.

46 of 164 adult patients with indolent systemic mastocytosis.

47 s, and the relationship of these findings to mastocytosis.

48 of cutaneous manifestations in patients with mastocytosis.

49 d digestive inflammation among patients with mastocytosis.

50 with and without elevated sBT levels and/or mastocytosis.

51 of cutaneous manifestations in patients with mastocytosis.

52 ciated with increased IL-6 levels, including mastocytosis.

53 bserved between disease subgroups: cutaneous mastocytosis (0.042%), indolent SM (0.285%), smoldering

54 maculopapular cutaneous mastocytosis/plaque mastocytosis, 22 nodular mastocytosis, and nine diffuse

55 levels of AOPPs and AGEs in 34 patients with mastocytosis (23 CM/MIS and 11 SM) and 27 healthy contro

56 icacy population; 16 had aggressive systemic mastocytosis, 57 had systemic mastocytosis with an assoc

57 In 116 patients with systemic mastocytosis (89 patients with advSM fulfilling the stri

58 dden mice promote rather than suppress focal mastocytosis, a critical tumor-promoting inflammatory re

59 gic patients with elevated sBT levels and/or mastocytosis, a panel of yellow jacket and honeybee veno

60 39 patients (age 17-90 years) diagnosed with mastocytosis according to WHO criteria between Jan 12, 1

61 years) with indolent or smouldering systemic mastocytosis, according to WHO classification or documen

62 ) is a very rare form of aggressive systemic mastocytosis accounting for < 1% of all mastocytosis.

63 Advanced systemic mastocytosis (advSM) is characterized by presence of the

64 In advanced systemic mastocytosis (advSM), disease evolution is often trigger

65 tients with cutaneous or non-severe systemic mastocytosis after a protocol amendment.

66 We included 21 adults with systemic mastocytosis and 18 healthy controls.

67 patients with indolent and advanced forms of mastocytosis and 20 healthy control subjects were enroll

68 Sixty-three patients had mastocytosis and 52 had reactions to previous hymenopter

69 biopsies from 9 of 10 patients with systemic mastocytosis and activating c-KIT mutations.

70 uous signaling via oncogenic KIT in systemic mastocytosis and acute myelogenous leukemia are poorly u

71 ith poor prognosis in patients with systemic mastocytosis and acute myeloid leukemia (AML).

72 is is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants

73 ad to better treatments for diseases such as mastocytosis and asthma.

74 f well defined prognostic groups in systemic mastocytosis and clarification of the merits of conventi

75 as enhanced toward NGF-beta gradient in both mastocytosis and controls.

76 e primary source of IL-9 to drive intestinal mastocytosis and experimental IgE-mediated food allergy.

77 te the management of patients with suspected mastocytosis and help avoid unnecessary referrals and in

78 n enhanced MC response in some patients with mastocytosis and in cases in which there is a greater in

79 Th1 and Th2 cytokines, and patterns of ileal mastocytosis and intestinal permeability.

80 first disease-specific QoL questionnaire for mastocytosis and is complemented by the MSAF, a short an

81 MD reduction, is detectable in patients with mastocytosis and is more severe in patients with high tr

82 eukin-4 (IL-4), a key mediator of intestinal mastocytosis and macromolecular permeability.

83 risk and impact of COVID-19 in patients with mastocytosis and mast cell activation syndromes.

84 osis variants, including aggressive systemic mastocytosis and mast cell leukemia.

85 The patient with systemic mastocytosis and MDS carried both mutated U2AF35 and KIT

86 -up for clonal MC disorders such as systemic mastocytosis and monoclonal MC activation syndrome were

87 Within a population of patients with mastocytosis and previous stings, we studied by BAT and

88 At the same time, the classification of mastocytosis and related diagnostic criteria have been r

89 Systemic mastocytosis and senior age are major, unmodifiable long

90 may appear de novo or secondary to previous mastocytosis and shares more clinicopathologic aspects w

91 luable tool for studying the pathogenesis of mastocytosis and should facilitate the development of no

92 on the frequency of drug hypersensitivity in mastocytosis and vice versa are scarce and evidence for

93 Fifty-three patients with mastocytosis and/or elevated sBT tryptase level and syst

94 al mast cell activation syndrome or systemic mastocytosis) and thus candidates for a bone marrow biop

95 sorders, type 2 diabetes, chronic urticaria, mastocytosis, and cancer.

96 isease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors.

97 intestinal infection by T. spiralis induces mastocytosis, and mast cell degranulation occurs when ch

98 mastocytosis/plaque mastocytosis, 22 nodular mastocytosis, and nine diffuse cutaneous mastocytosis -

99 ous lesions in patients with childhood-onset mastocytosis are associated with other disease parameter

100 life, and survival of patients with advanced mastocytosis are expected to improve in the coming years

101 Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass loca

102 rointestinal manifestations in patients with mastocytosis are highly prevalent and often severe.

103 Clinical manifestations of mastocytosis are mostly due to release of mediators from

104 Providers caring for patients with mastocytosis are tasked with the decision to consider th

105 ogic mast cells encountered in most forms of mastocytosis are unreliable in MCL.

106 licitor of the reaction, age of the patient, mastocytosis as comorbidity, severity of the reaction, a

107 , were significantly higher in patients with mastocytosis as compared to healthy controls.

108 ers such as urticaria, type I allergies, and mastocytosis as well as autoimmune and other inflammator

109 nd the pathogenesis of digestive features in mastocytosis as well as in other mast cell-mediated dise

110 The excess of MC in mastocytosis as well as the increased releasability of M

111 Aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL) are adva

112 Prognosis in systemic mastocytosis associated with another myeloid malignancy

113 Mastocytosis associated with germline KIT activating mut

114 reported to be increased in association with mastocytosis, asthma, and urticaria, is used in conjunct

115 ccording to WHO classification or documented mastocytosis based on histological criteria, at 50 centr

116 r, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of dis

117 unct in helping identify those with systemic mastocytosis but not monoclonal mast cell activation syn

118 ologic lesions were present in patients with mastocytosis but were not correlated with clinical sympt

119 approximately 29% of patients with systemic mastocytosis, but their pathogenetic or treatment releva

120 skin lesions are found and the diagnosis of mastocytosis can be established.

121 for patients with non-advanced and advanced mastocytosis can be used to predict survival outcomes an

122 onic secretion of mediators in patients with mastocytosis can lead to alteration of endothelial funct

123 thologic features of a patient with systemic mastocytosis caused by a de novo germline KIT K509I muta

124 on of APcK110, a novel Kit inhibitor, in the mastocytosis cell line HMC1.2 (KITV560G and KITD816V), a

125 us, it remains a potent inhibitor of AML and mastocytosis cell lines and primary AML samples.

126 ein and inhibited colony-forming capacity of mastocytosis cell lines.

127 clonal MC disorders in patients seen at our mastocytosis center with Hymenoptera sting-induced anaph

128 Mastocytosis, characterized by pathologic accumulation o

129 entation in diagnostic algorithms and future mastocytosis classifications is recommended.

130 a classification and criteria for cutaneous mastocytosis (CM) have been proposed, there remains a ne

131 Pediatric patients usually have cutaneous mastocytosis (CM) with mast cell infiltrates limited to

132 s stable and comparable to that of cutaneous mastocytosis (CM).

133 of mast cells limited to the skin (cutaneous mastocytosis: CM and mastocytosis in the skin: MIS) and/

134 gnificantly higher in D816V(+) patients with mastocytosis compared with D816V- patients or healthy co

135 Advanced systemic mastocytosis comprises rare hematologic neoplasms that a

136 n 16 of 22 patients, a diagnosis of indolent mastocytosis could be established, and 1 patient had a m

137 ld be biopsied and, as with the approach for mastocytosis diagnosis in the bone marrow, MC should be

138 Germline KIT mutations associated with mastocytosis drive a well-differentiated mast cell pheno

139 gistry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.

140 iciency, which is associated with intestinal mastocytosis, elevated levels of histamine, and enhanced

141 nfiltrating the bone marrow of patients with mastocytosis expressed TRAIL-R1.

142 Patients with mastocytosis featured elevated serum levels of NGF, NT-3

143 rolled 33 adults with cutaneous and systemic mastocytosis found 4 weeks of treatment with the second-

144 th non-advanced disease and 49 with advanced mastocytosis from the validation cohort.

145 Patients with mastocytosis had a significantly higher incidence of per

146 Patients with mastocytosis had higher SBT levels (P = .03) but only ra

147 Patients with mastocytosis had lower FMD compared with healthy control

148 in metabolites in the urine of patients with mastocytosis has not been critically examined in a large

149 Gastrointestinal manifestations of systemic mastocytosis have been previously studied in small cohor

150 rstanding of the pathophysiology of systemic mastocytosis have provided new therapeutic consideration

151 In patients with mastocytosis, HVAn prevalence does not increase constant

152 tion of baseline serum tryptase (sBT) and/or mastocytosis in about 5% of patients.

153 Mastocytosis in adults is associated with a history of a

154 s might contribute to the pathophysiology of mastocytosis in autocrine and paracrine loops.

155 or reversal of L-arginine deficiency blunts mastocytosis in ileal villi as well as bacterial translo

156 The prevalence of mastocytosis in patients with Hymenoptera venom allergy

157 ght to examine clinical aspects of pediatric mastocytosis in relationship to serum tryptase levels an

158 t of allergic diarrhea; they did not develop mastocytosis in the jejunum and had reduced ovalbumin-im

159 C mediator release symptoms in children with mastocytosis in the skin (MIS).

160 lar features of a cohort of 33 patients with mastocytosis in the skin in association with BM infiltra

161 Thirty-three patients with mastocytosis in the skin plus BM aggregates of round, fu

162 In adult patients with typical lesions of mastocytosis in the skin, a bone marrow (BM) biopsy shou

163 to the skin (cutaneous mastocytosis: CM and mastocytosis in the skin: MIS) and/or involving internal

164 cell growth factor KIT gene cause cutaneous mastocytosis in young children and systemic mastocytosis

165 gistry of the European Competence Network on Mastocytosis including 1639 patients (age 17-90 years) d

166 vailable for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem ce

167 pdate on prognosis and treatment of systemic mastocytosis, including investigational drug therapy.

168 efficacy in patients with advanced systemic mastocytosis, including the highly fatal variant mast-ce

169 Indolent systemic mastocytosis, including the subvariant of smouldering sy

170 The WHO classification separates mastocytosis into distinct variants, but prognostication

171 ng system divided patients with non-advanced mastocytosis into three groups: low (no risk factors), i

172 s International Prognostic Scoring System in Mastocytosis (IPSM) with data of 462 patients (age 17-79

173 Mastocytosis is a clonal disorder characterized by the p

174 Systemic mastocytosis is a clonal disorder of mast cells that may

175 Systemic mastocytosis is a clonal mast cell (MC) disease that can

176 Systemic mastocytosis is a hematological disease in which aberran

177 Mastocytosis is a heterogeneous disease characterized by

178 Mastocytosis is a heterogeneous disease characterized by

179 Mastocytosis is a heterogeneous disease characterized by

180 Systemic mastocytosis is a neoplastic disease of mast cells that

181 Mastocytosis is a rare disease characterized by clonal p

182 Mastocytosis is a rare heterogeneous disease characteriz

183 Mastocytosis is a term used to denote a heterogeneous gr

184 Smoldering mastocytosis is a variant with high systemic mast cell b

185 Mastocytosis is an emerging differential diagnosis in pa

186 Our data show that mastocytosis is associated with a state of increased oxi

187 Mastocytosis is characterized by clonal proliferation of

188 Mastocytosis is characterized by the accumulation of mas

189 Mastocytosis is frequently associated with mast cell-med

190 parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic m

191 uding the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with re

192 Clinical manifestations of indolent systemic mastocytosis (ISM) comprise mediator-related symptoms, a

193 Risk indicators of indolent systemic mastocytosis (ISM) in adults with clinical suspicion of

194 Indolent systemic mastocytosis (ISM) is a rare disease characterized by ac

195 Indolent systemic mastocytosis (ISM) patients have a normal life expectanc

196 esent in a subset of adult indolent systemic mastocytosis (ISM) patients in association with a poorer

197 Indolent systemic mastocytosis (ISM) without skin lesions (ISMs(-)) shows

198 requently in patients with indolent systemic mastocytosis (ISM), even before 50 years of age.

199 In indolent systemic mastocytosis (ISM), several risk factors of disease prog

200 patients suspected to have indolent systemic mastocytosis (ISM).

201 o of these individuals had indolent systemic mastocytosis (ISM).

202 gardless of the subtype of advanced systemic mastocytosis, KIT mutation status, or exposure to previo

203 ts, for example, into urticaria, angioedema, mastocytosis, led to the development of new therapeutic

204 mination, physical examination for cutaneous mastocytosis lesions, and clinical characteristics of an

205 ent clone, ROSA(KIT D816V), which produced a mastocytosis-like disease in NSG mice.

206 Mastocytosis (M) is a clonal myeloid-disabling disorder

207 ients presented with maculopapular cutaneous mastocytosis (MPCM), 12 patients presented with diffuse

208 astrointestinal histology from patients with mastocytosis (n = 23), control subjects with inflammator

209 secutive patients with different subtypes of mastocytosis (n = 329) admitted to the University Medica

210 d gastrointestinal symptoms of patients with mastocytosis (n = 83) were compared with those of matche

211 For patients with non-advanced mastocytosis (n=1380), we identified age 60 years or old

212 In patients with advanced mastocytosis (n=259), age 60 years or older (HR 2.14, 95

213 arrhea but surprisingly were not impaired in mastocytosis or allergen-specific immunoglobulin E.

214 ry features suggest the presence of 'occult' mastocytosis or another haematologic neoplasm, a BM inve

215 of KIT D816V and other signs or symptoms of mastocytosis or another haematopoietic disease, no BM in

216 h diffuse cutaneous (P < .0001) and systemic mastocytosis (P < .0001) and in all 3 categories versus

217 w mast cell burden in patients with systemic mastocytosis (P < .0001).

218 nases that are involved in indolent systemic mastocytosis pathogenesis.

219 , DAO concentrations were not elevated in 29 mastocytosis patients compared to healthy volunteers and

220 CCL2 levels were significantly increased in mastocytosis patients compared with controls.

221 We studied tryptophan metabolism in mastocytosis patients displaying or not gastrointestinal

222 amine degradation rate of DAO in plasma from mastocytosis patients during anaphylaxis is severely com

223 The perioperative management of mastocytosis patients is nontrivial; a multidisciplinary

224 Mastocytosis patients with digestive symptoms displayed

225 activity as compared to healthy controls and mastocytosis patients without digestive symptoms.

226 Mastocytosis patients without skin involvement pose a di

227 16V mutation of Kit, seen in the majority of mastocytosis patients, causes a robust activation of PI3

228 During severe anaphylaxis in mastocytosis patients, DAO is likely released from hepar

229 We now show, using a large cohort of mastocytosis patients, including an almost equal number

230 d the usefulness of BAT in subpopulations of mastocytosis patients, including those with negative tes

231 s with aberrant skin MC should be handled as mastocytosis patients.

232 rrelation between DAO and tryptase levels in mastocytosis patients.

233 riteria to avoid a misdiagnosis of cutaneous mastocytosis per current World Health Organization crite

234 ildren with MIS - 80 maculopapular cutaneous mastocytosis/plaque mastocytosis, 22 nodular mastocytosi

235 In the indolent systemic mastocytosis population, all mast cell load markers were

236 The management of children with pediatric mastocytosis poses a challenge.

237 D816V burden correlates with the variant of mastocytosis, predicts survival, and is a valuable follo

238 eous and indolent systemic mastocytosis, the Mastocytosis Quality of Life Questionnaire (MC-QoL).

239 ort on the development and validation of the mastocytosis quality-of-life questionnaire (MQLQ) and th

240 portions of KIT(+) ILC2s among patients with mastocytosis, regardless of D816V status.

241 relevant factor in identifying patients with mastocytosis, regardless of their serum tryptase levels.

242 twice daily in 116 patients, of whom 89 with mastocytosis-related organ damage were eligible for incl

243 complete resolution of at least one type of mastocytosis-related organ damage.

244 as well as in children with grade 4 (severe mastocytosis-related symptoms requiring emergency therap

245 ble role for ILC2s in the pathophysiology of mastocytosis remains unexplored.

246 indicator of severe anaphylaxis and possibly mastocytosis, requiring determination of BST.

247 this first comprehensive trial of a sgAH in mastocytosis, rupatadine 20 mg daily for 4 weeks signifi

248 Thus, a contribution of NTs to mastocytosis seems highly conceivable but has not yet be

249 outcome in 342 adult patients with systemic mastocytosis seen at our institution.

250 ssed in mast cells in normal, psoriasis, and mastocytosis skin as well as in bone marrow mast cells i

251 ssed in mast cells in normal, psoriasis, and mastocytosis skin.

252 clonal mast cell disorders (cMCD), systemic mastocytosis (SM) and monoclonal mast cell activation sy

253 ng to the clinical heterogeneity of systemic mastocytosis (SM) and to suboptimal responses to diverse

254 Cohorts with systemic mastocytosis (SM) and venom as well as idiopathic anaphy

255 at majority (>90%) of patients with systemic mastocytosis (SM) carry a common genetic lesion, the D81

256 cytosis (WDSM) is a rare variant of systemic mastocytosis (SM) characterized by bone marrow (BM) infi

257 Systemic mastocytosis (SM) has greatly benefited from the broad a

258 4.4 (SD 43.2) in five patients with systemic mastocytosis (SM) in comparison with autopsy skin (39.1

259 Systemic mastocytosis (SM) is characterized by abnormal accumulat

260 Systemic mastocytosis (SM) is characterized by accumulation of ne

261 Systemic mastocytosis (SM) may be associated with hymenoptera all

262 Patients with systemic mastocytosis (SM) may suffer from mast cell (MC) mediato

263 Indolent systemic mastocytosis (SM) patients have a varied clinical presen

264 Advanced systemic mastocytosis (SM), a fatal hematopoietic malignancy char

265 In systemic mastocytosis (SM), clinical problems arise from factor-i

266 lls (MCs) in patients with advanced systemic mastocytosis (SM).

267 sent in a majority of patients with systemic mastocytosis (SM).

268 row (BM) investigation in suspected systemic mastocytosis (SM).

269 ) and/or involving internal organs (systemic mastocytosis: SM).

270 Despite the good prognosis of pediatric mastocytosis, some patients suffer from severe mast cell

271 quality-of-life questionnaire (MQLQ) and the mastocytosis symptom assessment form (MSAF).

272 he MSAF reveals that fatigue is the severest mastocytosis symptom while the MQLQ indicates that fear

273 d rupatadine 20 mg daily in the treatment of mastocytosis symptoms in 30 adult patients.

274 ion (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast

275 uently and were more severe in patients with mastocytosis than in healthy subjects: bloating (33% vs

276 more clinicopathologic aspects with systemic mastocytosis than with acute myeloid leukemia.

277 gressive and nonaggressive cases of systemic mastocytosis, that in contrast to the oncogenic KITD816V

278 atients with cutaneous and indolent systemic mastocytosis, the Mastocytosis Quality of Life Questionn

279 In patients with mastocytosis, the negative results of standard tests are

280 isk patients with elevated sBT levels and/or mastocytosis, the use of molecular components and decrea

281 including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma &

282 tigate endothelial function in patients with mastocytosis using a noninvasive technique of flow-media

283 was not as good in the WHO indolent systemic mastocytosis variant of smoldering mastocytosis.

284 systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mas

285 Although mastocytosis was absent, the protease unique to mucosal

286 Mastocytosis was diagnosed in 7.7% of adult patients and

287 Life expectancy in indolent systemic mastocytosis was not significantly different than that o

288 Well-differentiated systemic mastocytosis (WDSM) is a rare variant of systemic mastoc

289 Analogous to the criteria for mastocytosis, we suggest a skin score criteria including

290 m healthy control subjects and patients with mastocytosis were assayed for IL-6, tryptase, and sIL-6R

291 Novel biomarkers and treatment for mastocytosis were presented in several studies.

292 lar mastocytosis, and nine diffuse cutaneous mastocytosis - were investigated as a predictive biomark

293 MMC9 induction failed to develop intestinal mastocytosis, which resulted in decreased food allergy s

294 In patients with indolent systemic mastocytosis with a history of Hymenoptera venom exposur

295 mastocytosis in young children and systemic mastocytosis with a more guarded prognosis in adults who

296 ssive systemic mastocytosis, 57 had systemic mastocytosis with an associated hematologic neoplasm, an

297 ld Health Organization category of "systemic mastocytosis with associated clonal hematologic nonmast

298 ese variables, a separate score for advanced mastocytosis with four risk categories was established,

299 leukemia cases and in 1 child with systemic mastocytosis with MDS.

300 overview of recent advances in the field of mastocytosis, with emphasis on classification, prognosti