ライフサイエンスコーパス: mastocytosis

1 ed cold urticaria, mosquito bite allergy and mastocytosis.

2 g new treatment options in advanced systemic mastocytosis.

3 cacy of cladribine in 68 adult patients with mastocytosis.

4 assessed in those patients with ascertained mastocytosis.

5 eases associated with KIT mutations, such as mastocytosis.

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

7 nfiltrating the bone marrow of patients with mastocytosis.

8 ow and peripheral blood of 105 patients with mastocytosis.

9 nostic algorithm for patients with suspected mastocytosis.

10 e myeloid leukemia, testicular seminomas and mastocytosis.

11 gastrointestinal symptoms for patients with mastocytosis.

12 rointestinal manifestations in patients with mastocytosis.

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

14 T levels might also indicate the presence of mastocytosis.

15 and TrkC on intestinal MCs of patients with mastocytosis.

16 h increasing mast cell load in patients with mastocytosis.

17 allergies, other inflammatory reactions, and mastocytosis.

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

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

20 for severe MC activation events in pediatric mastocytosis.

21 drug resistance in neoplastic MC in advanced mastocytosis.

22 rker to diagnose and predict severe forms of mastocytosis.

23 nd in the majority of patients with systemic mastocytosis.

24 WHO, and the European Competence Network on Mastocytosis.

25 f conventional drugs for aggressive systemic mastocytosis.

26 ciated with the myeloproliferative disorder, mastocytosis.

27 the suppression of mast cell disease such as mastocytosis.

28 systemic mastocytosis variant of smoldering mastocytosis.

29 vity in serum of most subjects with systemic mastocytosis.

30 had evidence of 1 or more minor criteria for mastocytosis.

31 al mast-cell aggregates observed in systemic mastocytosis.

32 alignant cells in an in vivo murine model of mastocytosis.

33 ngly correlated with tissue eosinophilia and mastocytosis.

34 ays an important role in the pathogenesis of mastocytosis.

35 that is indicative of an underlying systemic mastocytosis.

36 e in granuloma size, tissue eosinophilia, or mastocytosis.

37 rointestinal (GI) stromal tumors (GISTs) and mastocytosis.

38 symptomatic indolent or smouldering systemic mastocytosis.

39 of 164 adult patients with indolent systemic mastocytosis.

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

41 marrow mast cells in patients with systemic mastocytosis.

42 of cutaneous manifestations in patients with mastocytosis.

43 d digestive inflammation among patients with mastocytosis.

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

45 of cutaneous manifestations in patients with mastocytosis.

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

47 e MQLQ and MSAF with independent measures of mastocytosis.

48 atients with cutaneous and indolent systemic mastocytosis.

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

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

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

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

53 gnificant determinant of HRQoL impairment in mastocytosis.

54 correlate well with bone marrow findings of mastocytosis.

55 articularly in patients with childhood-onset mastocytosis.

56 n associated with several diseases including mastocytosis.

57 ersistent disease resembling adulthood-onset mastocytosis.

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

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

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

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

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

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

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

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

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

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

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 p, most commonly D816V, are seen in systemic mastocytosis and acute myelogenous leukemia.

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

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

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

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

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

77 r a clonal disorder of mast cells related to mastocytosis and for which novel targeted therapies migh

78 , KIT protein, and are associated with human mastocytosis and gastrointestinal stromal tumors (GISTs)

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

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

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

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

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

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

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

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

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

88 lular biology of mast cells in patients with mastocytosis and those of healthy individuals.

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

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

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

92 ciated with gastrointestinal stromal tumors, mastocytosis, and acute myeloid leukemia.

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

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

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

96 16 that are typically found in AML, systemic mastocytosis, and seminoma are insensitive to imatinib m

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

98 The symptoms of systemic mastocytosis are due to the pathologic accumulation and

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

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

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

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

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

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

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

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

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

108 atment options exist for aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL).

109 disease similar to human aggressive systemic mastocytosis (ASM) or mast cell leukemia (MCL) when they

110 Prognosis in systemic mastocytosis associated with another myeloid malignancy

111 Mastocytosis associated with germline KIT activating mut

112 s no effective curative therapy for systemic mastocytosis associated with KITD816V.

113 effective, in vitro and in vivo, against the mastocytosis-associated c-kit D816V mutation.

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

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

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

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

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

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

122 at (a) APcK110 inhibits proliferation of the mastocytosis cell line HMC1.2 and the SCF-responsive cel

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

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

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

126 Mastocytosis, characterized by pathologic accumulation o

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

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

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

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

131 Advanced systemic mastocytosis comprises rare hematologic neoplasms that a

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

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

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

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

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

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

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

139 reatment of c-kit-related diseases including mastocytosis, GISTs, mast cell leukemia, subtypes of acu

140 Patients with mastocytosis had a significantly higher incidence of per

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

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

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

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

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

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

147 Mastocytosis in adults is associated with a history of a

148 ansmembrane c-kit mutations in patients with mastocytosis in association with the decision to use ima

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

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

151 to CMML and AML, we found that NRAS induced mastocytosis in mice.

152 sponse and pronounced intestinal and splenic mastocytosis in mice.

153 The prevalence of mastocytosis in patients with Hymenoptera venom allergy

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

155 t of exaggerated goblet cell hyperplasia and mastocytosis in the gut due to increased production of T

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

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

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

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

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

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

162 hanced parasite-specific IgG1 and intestinal mastocytosis in vivo, leading to accelerated expulsion o

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

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

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

166 Indolent systemic mastocytosis, including the subvariant of smouldering sy

167 astic mast cells isolated from patients with mastocytosis, incubated with 17-AAG ex vivo, are selecti

168 intestinal anaphylaxis phenotype (intestinal mastocytosis, intestinal permeability, and intravascular

169 Mastocytosis is a clonal disorder characterized by the p

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

171 Systemic mastocytosis is a clonal disorder of the mast cell and i

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

173 Mastocytosis is a heterogeneous disease characterized by

174 Mastocytosis is a heterogeneous disease characterized by

175 Mastocytosis is a heterogeneous disease characterized by

176 Systemic mastocytosis is a neoplastic disease of mast cells that

177 Mastocytosis is a rare heterogeneous disease characteriz

178 Mastocytosis is a term used to denote a heterogeneous gr

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

180 Mastocytosis is an emerging differential diagnosis in pa

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

182 Mastocytosis is associated with an activating mutation i

183 In hematology, the clinical disease of mastocytosis is characterized by a pathologic increase o

184 Mastocytosis is characterized by clonal proliferation of

185 Mastocytosis is characterized by the accumulation of mas

186 Mastocytosis is frequently associated with mast cell-med

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

188 Skin-only disease (cutaneous mastocytosis) is infrequent in adults and systemic masto

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

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

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

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

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

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

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

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

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

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

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

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

201 urine Kit (mKit)] are found in patients with mastocytosis, leukemia, and germ cell tumors.

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

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

204 ytosis) is infrequent in adults and systemic mastocytosis may be broadly classified as an indolent or

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

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

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

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

209 The splenic mastocytosis observed in BALB/c mice following infection

210 of peripheral blood eosinophilia and jejunal mastocytosis occurred in wild-type and IgE-deficient ani

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

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

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

214 an occur in the absence of either intestinal mastocytosis or RMCPII release.

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

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

217 nases that are involved in indolent systemic mastocytosis pathogenesis.

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

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

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

221 Mastocytosis patients with digestive symptoms displayed

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

223 Mastocytosis patients without skin involvement pose a di

224 16V mutation of Kit, seen in the majority of mastocytosis patients, causes a robust activation of PI3

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

240 Reactive mastocytosis (RM) in epithelial surfaces is a consistent

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

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

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

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

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

246 kinase activity are associated with systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL

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

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

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

250 4.4 (SD 43.2) in five patients with systemic mastocytosis (SM) in comparison with autopsy skin (39.1

251 Systemic mastocytosis (SM) is characterized by abnormal accumulat

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

253 Clinical phenotype in systemic mastocytosis (SM) is markedly variable, which complicate

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

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

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

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

258 eosinophilic syndrome (HES), 8 with systemic mastocytosis (SM), and 6 with chronic neutrophilic leuke

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

260 y a key role in the pathogenesis of systemic mastocytosis (SM), gastrointestinal stromal tumors (GIST

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

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

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

264 ute myelogenous leukemia (AML), and systemic mastocytosis (SM).

265 in acute myeloid leukemia (AML) and systemic mastocytosis (SM); however, unlike the KIT juxtamembrane

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

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

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

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

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

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

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

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

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

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

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

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

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

279 ents with blood eosinophilia and bone marrow mastocytosis; this mutation has since joined several oth

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

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

282 Although mastocytosis was absent, the protease unique to mucosal

283 Mastocytosis was diagnosed in 7.7% of adult patients and

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

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

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

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

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

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

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

291 arrow mast cells from patients with systemic mastocytosis while sparing other hematopoietic cells.

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

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

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

295 specific entities, such as SM-MPN, systemic mastocytosis with chronic myelomonocytic leukemia, SM-MD

296 A positive chronic eosinophilic leukemia and mastocytosis with imatinib.

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

298 yelomonocytic leukemia, SM-MDS, and systemic mastocytosis with-acute leukemia, rather than their broa

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

300 o the muscle phase did not induce intestinal mastocytosis, yet such rats exhibited rapid expulsion wh