ライフサイエンスコーパス: hemophagocytic

1 by preventing macrophages from becoming both hemophagocytic and hyperactivated under proinflammation.

2 est that a variety of triggers can result in hemophagocytic disease.

3 nd autoimmune lymphoproliferative syndromes, hemophagocytic disorders, lymphoproliferative diseases,

4 vation syndrome and other clinically similar hemophagocytic disorders.

5 Consistent with this observation, hemophagocytic histiocytes from patients with macrophage

6 We show that primary macrophages become hemophagocytic in the absence or presence of IFN-gamma u

7 lation leading to widespread lymphocytic and hemophagocytic infiltration of vital organs.

8 nism(s) that triggers a macrophage to become hemophagocytic is unknown, but it has been reported that

9 surveillance and leads to the development of hemophagocytic lymphohistiocytic syndrome (HLH).

10 Hemophagocytic lymphohistiocytoses represent a rare but

11 e molecular and pathophysiologic features of hemophagocytic lymphohistiocytoses.

12 of patients with both primary and secondary hemophagocytic lymphohistiocytoses.

13 yperferritinemia, 40 were considered to have hemophagocytic lymphohistiocytosis (1.52%).

14 ed odds ratio, 1.86; 95% CI, 1.15-2.99), and hemophagocytic lymphohistiocytosis (adjusted odds ratio,

15 ed odds ratio, 3.54; 95% CI, 1.80-6.95), and hemophagocytic lymphohistiocytosis (adjusted odds ratio,

16 92.5% sensitivity and 91.9% specificity for hemophagocytic lymphohistiocytosis (area under the curve

17 Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a fatal

18 in males with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH).

19 Familial hemophagocytic lymphohistiocytosis (F-HLH) and Griscelli

20 HLH occurs as both acquired and familial hemophagocytic lymphohistiocytosis (FHL) forms.

21 Familial hemophagocytic lymphohistiocytosis (FHL) is a geneticall

22 Familial hemophagocytic lymphohistiocytosis (FHL) is a life-threa

23 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare and o

24 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, gene

25 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, rapi

26 Familial hemophagocytic lymphohistiocytosis (FHL) is an inherited

27 Familial hemophagocytic lymphohistiocytosis (FHL) is caused by ge

28 f PBMCs obtained from patients with familial hemophagocytic lymphohistiocytosis (FHL) to screen for b

29 Cytokine storm syndromes, such as familial hemophagocytic lymphohistiocytosis (FHL), are lethal dis

30 of patients, referred to as having familial hemophagocytic lymphohistiocytosis (FHL), have various u

31 molecules have been associated with familial hemophagocytic lymphohistiocytosis (FHL).

32 ein syntaxin 11 result in a form of familial hemophagocytic lymphohistiocytosis (FHL).

33 -4, in which mutations cause type 3 familial hemophagocytic lymphohistiocytosis (FHL3), a fatal disea

34 (NK) cytotoxicity, and give rise to familial hemophagocytic lymphohistiocytosis (FHL4 or FHL5, respec

35 ive disorders collectively known as familial hemophagocytic lymphohistiocytosis (FHLH), all associate

36 cells and developed all clinical symptoms of hemophagocytic lymphohistiocytosis (HLH) after infection

37 ive T-cell activation and the fatal disorder hemophagocytic lymphohistiocytosis (HLH) after infection

38 ed to be associated with a high incidence of hemophagocytic lymphohistiocytosis (HLH) and a lack of l

39 Hemophagocytic lymphohistiocytosis (HLH) and macrophage

40 The clinical syndromes of hemophagocytic lymphohistiocytosis (HLH) and macrophage

41 Hemophagocytic lymphohistiocytosis (HLH) and macrophage

42 he hyperinflammatory state characteristic of hemophagocytic lymphohistiocytosis (HLH) and macrophage

43 forms of the severe immunoregulatory disease hemophagocytic lymphohistiocytosis (HLH) arise from bial

44 poietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) at the cost of

45 Primary hemophagocytic lymphohistiocytosis (HLH) can be caused b

46 e small ssRNAs and develop spontaneous fatal hemophagocytic lymphohistiocytosis (HLH) characterized b

47 Hemophagocytic lymphohistiocytosis (HLH) comprises a sev

48 Hemophagocytic lymphohistiocytosis (HLH) comprises an em

49 eneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is as

50 tients with primary and infection-associated hemophagocytic lymphohistiocytosis (HLH) from patients w

51 Hemophagocytic lymphohistiocytosis (HLH) is a devastatin

52 Hemophagocytic lymphohistiocytosis (HLH) is a disorder t

53 Hemophagocytic lymphohistiocytosis (HLH) is a fatal diso

54 Hemophagocytic lymphohistiocytosis (HLH) is a hyperinfla

55 Hemophagocytic lymphohistiocytosis (HLH) is a hyperinfla

56 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

57 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

58 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

59 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

60 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

61 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

62 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

63 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

64 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

65 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

66 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

67 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

68 Hemophagocytic lymphohistiocytosis (HLH) is a potentiall

69 Hemophagocytic lymphohistiocytosis (HLH) is a rare disor

70 Hemophagocytic lymphohistiocytosis (HLH) is a rare disor

71 Hemophagocytic lymphohistiocytosis (HLH) is a rare infla

72 Hemophagocytic lymphohistiocytosis (HLH) is a rare life-

73 Familial hemophagocytic lymphohistiocytosis (HLH) is a rare prima

74 Hemophagocytic lymphohistiocytosis (HLH) is a rare syndr

75 Hemophagocytic lymphohistiocytosis (HLH) is a rare, life

76 Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a rare, life

77 Hemophagocytic lymphohistiocytosis (HLH) is a severe inf

78 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome d

79 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome o

80 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome o

81 Hemophagocytic lymphohistiocytosis (HLH) is a systemic h

82 Hemophagocytic lymphohistiocytosis (HLH) is an immune dy

83 Hemophagocytic lymphohistiocytosis (HLH) is an inborn di

84 Hemophagocytic lymphohistiocytosis (HLH) is an inflammat

85 Hemophagocytic lymphohistiocytosis (HLH) is an interfero

86 Hemophagocytic lymphohistiocytosis (HLH) is an often-fat

87 Hemophagocytic lymphohistiocytosis (HLH) is characterize

88 Hemophagocytic lymphohistiocytosis (HLH) is characterize

89 imary, genetic forms from secondary forms of hemophagocytic lymphohistiocytosis (HLH) is crucial for

90 receptor (CAR) T-cell toxicities resembling hemophagocytic lymphohistiocytosis (HLH) occur in a subs

91 Frequently fatal, primary hemophagocytic lymphohistiocytosis (HLH) occurs in infan

92 ate-onset inflammatory toxicities resembling hemophagocytic lymphohistiocytosis (HLH) or macrophage a

93 iable clinical consequences, including fatal hemophagocytic lymphohistiocytosis (HLH) triggered predo

94 ns can improve the outcomes of patients with hemophagocytic lymphohistiocytosis (HLH) undergoing allo

95 Hemophagocytic lymphohistiocytosis (HLH) was only observ

96 of pediatric and adult patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory

97 Hemophagocytic lymphohistiocytosis (HLH), a disease ofte

98 ad set of triggering signals can precipitate hemophagocytic lymphohistiocytosis (HLH), a life-threate

99 evere COVID-19, with parallels proposed with hemophagocytic lymphohistiocytosis (HLH), a life-threati

100 Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset St

101 erferritinemia and cytopenias are present in hemophagocytic lymphohistiocytosis (HLH), and somatic mu

102 thologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some autho

103 2) inhibitor ruxolitinib in murine models of hemophagocytic lymphohistiocytosis (HLH), and the HLH-si

104 Cytokine storm syndromes (CSSs), including hemophagocytic lymphohistiocytosis (HLH), are increasing

105 t spontaneously develop clinical features of hemophagocytic lymphohistiocytosis (HLH), but do so when

106 ne have been described in some patients with hemophagocytic lymphohistiocytosis (HLH), but the role o

107 led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized

108 that can present with recurrent episodes of hemophagocytic lymphohistiocytosis (HLH), though the exa

109 It is clinically similar to hemophagocytic lymphohistiocytosis (HLH), which is cause

110 oss of normal immune regulation and underlie hemophagocytic lymphohistiocytosis (HLH), which requires

111 Here, we characterize 2 novel monoallelic hemophagocytic lymphohistiocytosis (HLH)-associated muta

112 mphocyte cytotoxicity predispose patients to hemophagocytic lymphohistiocytosis (HLH).

113 ls engaged by BiTE antibodies and leading to hemophagocytic lymphohistiocytosis (HLH).

114 rt than in their earlier reports of familial hemophagocytic lymphohistiocytosis (HLH).

115 develop a fatal inflammatory disorder called hemophagocytic lymphohistiocytosis (HLH).

116 al manifestations of hemophagocytic syndrome/hemophagocytic lymphohistiocytosis (HLH).

117 cterized hyperinflammatory syndromes such as hemophagocytic lymphohistiocytosis (HLH).

118 amatic hyperferritinemic inflammation termed hemophagocytic lymphohistiocytosis (HLH).

119 plete STAT2 protein abrogation presents with hemophagocytic lymphohistiocytosis (HLH).

120 and lymphoproliferative disorders, including hemophagocytic lymphohistiocytosis (HLH).

121 ndividuals and can have features of reactive hemophagocytic lymphohistiocytosis (HLH).

122 is often valuable in patients with acquired hemophagocytic lymphohistiocytosis (i.e., secondary hemo

123 e (n = 9), central nervous system-restricted hemophagocytic lymphohistiocytosis (n = 3), anti-GABA(A)

124 T) is the only curative treatment in primary hemophagocytic lymphohistiocytosis (pHLH).

125 Secondary hemophagocytic lymphohistiocytosis (sHLH) is a highly mo

126 Secondary hemophagocytic lymphohistiocytosis (sHLH) is a potential

127 A hallmark of secondary hemophagocytic lymphohistiocytosis (sHLH), a severe form

128 atal immune dysregulation disorder, familial hemophagocytic lymphohistiocytosis (type 2 FHL, FHL2).

129 Meeting five or more of eight hemophagocytic lymphohistiocytosis 2004 diagnostic crite

130 tify Munc13-4, a protein mutated in familial hemophagocytic lymphohistiocytosis 3, as a WPB-tethering

131 ty arose in 4 patients (2/4, grade 3-4), and hemophagocytic lymphohistiocytosis affected 2 patients.

132 chosocial outcomes in childhood survivors of hemophagocytic lymphohistiocytosis after hematopoietic s

133 nts with a clinically suspected diagnosis of hemophagocytic lymphohistiocytosis and found 28 patients

134 AKI is frequent in severe hemophagocytic lymphohistiocytosis and has been attribut

135 0.99-0.99) and 0.99 (95% CI, 0.99-0.99) for hemophagocytic lymphohistiocytosis and HScore, respectiv

136 ment is indicated for patients with familial hemophagocytic lymphohistiocytosis and is often valuable

137 Although it has been proposed that in both hemophagocytic lymphohistiocytosis and macrophage activa

138 as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activa

139 ic benefits in preclinical disease models of hemophagocytic lymphohistiocytosis and multiple sclerosi

140 -eating macrophages, is seen in the disorder hemophagocytic lymphohistiocytosis and other inflammator

141 In familial hemophagocytic lymphohistiocytosis and severe, persisten

142 yperinflammatory syndromes, however, such as hemophagocytic lymphohistiocytosis and the newly classif

143 atory phenomena such as hyperinflammation or hemophagocytic lymphohistiocytosis are a frequent yet pa

144 In summary, childhood survivors of hemophagocytic lymphohistiocytosis are at risk of long-t

145 ogic abnormalities in patients with familial hemophagocytic lymphohistiocytosis are decreased natural

146 haracterized by immunodeficiency and a fatal hemophagocytic lymphohistiocytosis caused by impaired fu

147 rve was then of 0.98 (95% CI, 0.96-0.99) for hemophagocytic lymphohistiocytosis criteria and 0.99 (95

148 nding factors, our study suggests HScore and hemophagocytic lymphohistiocytosis criteria to be highly

149 ic syndrome- patients (p < 0.001); number of hemophagocytic lymphohistiocytosis criteria was 4 (4-5)

150 Median number of hemophagocytic lymphohistiocytosis criteria was 4 (4-5)

151 Hemophagocytic lymphohistiocytosis diagnosis was based o

152 We analyze ferritin levels to differentiate hemophagocytic lymphohistiocytosis from other causes of

153 ive cytopenias, hyperferritinemia, and other hemophagocytic lymphohistiocytosis hallmarks were appare

154 about half of patients with severe forms of hemophagocytic lymphohistiocytosis have been developed.

155 n in low baseline severity of illness versus hemophagocytic lymphohistiocytosis in higher baseline se

156 ore, may contribute to improved diagnosis of hemophagocytic lymphohistiocytosis in ICU.

157 etermine optimal treatment for patients with hemophagocytic lymphohistiocytosis in ICUs, including th

158 Mortality of hemophagocytic lymphohistiocytosis in the ICU is high.

159 The better understood familial hemophagocytic lymphohistiocytosis is a constellation of

160 Hemophagocytic lymphohistiocytosis is a cytokine release

161 Hemophagocytic lymphohistiocytosis is a cytokine-driven

162 Hemophagocytic lymphohistiocytosis is a disease of abnor

163 Hemophagocytic lymphohistiocytosis is a hyperinflammator

164 Hemophagocytic lymphohistiocytosis is a hyperinflammator

165 Familial hemophagocytic lymphohistiocytosis is a life-threatening

166 Hemophagocytic lymphohistiocytosis is a life-threatening

167 Primary hemophagocytic lymphohistiocytosis is a rare syndrome ch

168 The hemophagocytic lymphohistiocytosis is probably underdiag

169 It does not cover EBV and hemophagocytic lymphohistiocytosis nor lymphomagenesis r

170 IFN-gamma is largely considered a driver of hemophagocytic lymphohistiocytosis pathology, IFN-gamma

171 igating treatment tailored to critically ill hemophagocytic lymphohistiocytosis patients are highly w

172 Maximum ferritin levels were highest in hemophagocytic lymphohistiocytosis patients compared wit

173 In approximately one third of familial hemophagocytic lymphohistiocytosis patients, these immun

174 ities, and mortality in critically ill adult hemophagocytic lymphohistiocytosis patients.

175 with the highest ferritin levels observed in hemophagocytic lymphohistiocytosis patients.

176 set pancytopenia and severe autoinflammation/hemophagocytic lymphohistiocytosis requiring emapalumab

177 y, including macrophage activation syndrome (hemophagocytic lymphohistiocytosis secondary to autoimmu

178 sis, frequently included in a postinfectious hemophagocytic lymphohistiocytosis setting.

179 n between macrophage activation syndrome and hemophagocytic lymphohistiocytosis still remain to be de

180 ny clinical and laboratory manifestations of hemophagocytic lymphohistiocytosis syndrome (HLS), also

181 anatory mechanisms, possibly integrated into hemophagocytic lymphohistiocytosis syndrome, of infectio

182 tural killer cell dysfunction leading to the hemophagocytic lymphohistiocytosis syndromes.

183 ead to a spectrum of diseases, from familial hemophagocytic lymphohistiocytosis to an increased risk

184 codes Munc13-4, are associated with familial hemophagocytic lymphohistiocytosis type 3 (FHL3).

185 Munc13-4, the mutation of which in familial hemophagocytic lymphohistiocytosis type 3 results in a p

186 We describe a child with familial hemophagocytic lymphohistiocytosis type 3 who developed

187 ted with the human immunodeficiency familial hemophagocytic lymphohistiocytosis type 3.

188 totoxic granule exocytosis, causing familial hemophagocytic lymphohistiocytosis type 4 (FHL-4).

189 n-knockout mouse strains and from a Familial Hemophagocytic Lymphohistiocytosis type 4 (FHL4) patient

190 ients with STX11 deficiency develop familial hemophagocytic lymphohistiocytosis type 4 (FHL4), a life

191 Familial hemophagocytic lymphohistiocytosis type 5 (FHL5) is caus

192 Recent analysis of patients with familial hemophagocytic lymphohistiocytosis type 5 has identified

193 Similarly, treating children with familial hemophagocytic lymphohistiocytosis using reduced intensi

194 A liver biopsy showed hemophagocytic lymphohistiocytosis with exuberant infilt

195 by inborn errors of immunity (i.e., familial hemophagocytic lymphohistiocytosis) or develop as a comp

196 gocytic lymphohistiocytosis (i.e., secondary hemophagocytic lymphohistiocytosis) without previous the

197 mutations account for 20% to 40% of familial hemophagocytic lymphohistiocytosis, a fatal disease of e

198 ng mutations in these genes develop familial hemophagocytic lymphohistiocytosis, a primary immunodefi

199 iated hydroa vacciniforme-like lymphoma with hemophagocytic lymphohistiocytosis, and 2 had EBV-positi

200 including some peripheral T-cell lymphomas, hemophagocytic lymphohistiocytosis, and chronic active E

201 rtality during SARS-CoV-2 infection, sepsis, hemophagocytic lymphohistiocytosis, and cytokine shock.

202 rtality during SARS-CoV-2 infection, sepsis, hemophagocytic lymphohistiocytosis, and cytokine shock.

203 and malignant lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, and smooth muscle an

204 were to identify the frequency of secondary hemophagocytic lymphohistiocytosis, and the main prognos

205 e for treating some cases of type 2 familial hemophagocytic lymphohistiocytosis, based on the pharmac

206 atients admitted with suspected or diagnosed hemophagocytic lymphohistiocytosis, between January 1, 2

207 Multiple prior malignancies, hemophagocytic lymphohistiocytosis, congenital immunodef

208 D, STX11, or STXBP2 associated with familial hemophagocytic lymphohistiocytosis, CTL and NK cell degr

209 tion, hyperinflammatory syndromes, including hemophagocytic lymphohistiocytosis, developed after SARS

210 re and often fatal infectious mononucleosis, hemophagocytic lymphohistiocytosis, lymphoproliferative

211 ncluding fulminant infectious mononucleosis, hemophagocytic lymphohistiocytosis, lymphoproliferative

212 , renal, hepatic, and neurologic toxicities, hemophagocytic lymphohistiocytosis, opportunistic infect

213 genetic disorders such as leukodystrophies, hemophagocytic lymphohistiocytosis, or genetic vasculopa

214 t cytopenia, autoinflammation, and recurrent hemophagocytic lymphohistiocytosis, p.C188Y and p.*192Ce

215 sttransplant plasma cell hepatitis, familial hemophagocytic lymphohistiocytosis, pediatric nonalcohol

216 In familial hemophagocytic lymphohistiocytosis, Prf-deficient infant

217 neurotoxicity, cytokine release syndrome and hemophagocytic lymphohistiocytosis, represented only a m

218 Patients were categorized into hemophagocytic lymphohistiocytosis, sepsis, septic shock

219 Ferritin levels in patients with hemophagocytic lymphohistiocytosis, sepsis, septic shock

220 ed lymphomas, lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, solid tumors, and ot

221 sociated with macrophage activation syndrome/hemophagocytic lymphohistiocytosis, such as interleukin

222 he perforin gene have been found in familial hemophagocytic lymphohistiocytosis, which shares some fe

223 c lymphohistiocytosis diagnosis was based on Hemophagocytic Lymphohistiocytosis-2004 criteria and the

224 irms the occurrence of reversible AKI due to hemophagocytic lymphohistiocytosis-induced activated mac

225 Hemophagocytic lymphohistiocytosis-like manifestations w

226 onse such as immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome may occ

227 One grade 2 immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome was obs

228 mental status and neurologic dysfunction, or hemophagocytic lymphohistiocytosis-macrophage-activation

229 ar to that of macrophage activation syndrome/hemophagocytic lymphohistiocytosis.

230 esults occurred only for infection-triggered hemophagocytic lymphohistiocytosis.

231 rimary immunodeficiencies including familial hemophagocytic lymphohistiocytosis.

232 ells, resulting in the development of lethal hemophagocytic lymphohistiocytosis.

233 e 18 years of age or younger and had primary hemophagocytic lymphohistiocytosis.

234 ppropriately classified as X-linked familial hemophagocytic lymphohistiocytosis.

235 equired to induce disease in mouse models of hemophagocytic lymphohistiocytosis.

236 g them are Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis.

237 y results in several human diseases, such as hemophagocytic lymphohistiocytosis.

238 des, T-cell lymphoproliferative disease, and hemophagocytic lymphohistiocytosis.

239 mutations in Munc13-4 as a cause of familial hemophagocytic lymphohistiocytosis.

240 with familial and virus-associated reactive hemophagocytic lymphohistiocytosis.

241 are associated with some cases of CAEBV with hemophagocytic lymphohistiocytosis.

242 ked lymphoproliferative disease and familial hemophagocytic lymphohistiocytosis.

243 he multisystem organ failure associated with hemophagocytic lymphohistiocytosis.

244 perforin have been reported in patients with hemophagocytic lymphohistiocytosis.

245 ion related to severely elevated ferritin is hemophagocytic lymphohistiocytosis.

246 AdV type 41, one of whom developed secondary hemophagocytic lymphohistiocytosis.

247 ria serves as a valuable diagnostic tool for hemophagocytic lymphohistiocytosis.

248 s targeted therapy for patients with primary hemophagocytic lymphohistiocytosis.

249 east five of the eight HLH-2004 criteria for hemophagocytic lymphohistiocytosis.

250 o those implicated in the setting of primary hemophagocytic lymphohistiocytosis.

251 escribed by examining patients with familial hemophagocytic lymphohistiocytosis.

252 erest in discussing a specific treatment for hemophagocytic lymphohistiocytosis.

253 ng examples from several diseases, including hemophagocytic lymphohistiocytosis/macrophage activation

254 infectious mononucleosis-like disease and/or hemophagocytic lymphohistocytosis (HLH), reflecting ecto

255 n VEO-IBD and can increase susceptibility to hemophagocytic lymphohistocytosis (HLH).

256 S. Typhimurium can reside within hemophagocytic macrophages (HMs) in SV129S6 mice, an Slc

257 Phenotypic characterization of hemophagocytic macrophages has been another focus of res

258 murium was recovered 24 h after infection of hemophagocytic macrophages in 2-fold-higher numbers than

259 extramedullary erythropoiesis, and increased hemophagocytic macrophages in the bone marrow, liver, an

260 ysiologic conditions that favor expansion of hemophagocytic macrophages provide a source of new MAS b

261 The anti-inflammatory phenotype of hemophagocytic macrophages suggests that these cells con

262 driven by excessive expansion of T cells and hemophagocytic macrophages.

263 ctivation and expansion of T lymphocytes and hemophagocytic macrophages.

264 only restrains macrophages from acquiring a hemophagocytic phenotype but also tempers their proinfla

265 Reactive hemophagocytic syndrome (44%), autoimmune hemolytic anem

266 toms resembling those seen in EBV-associated hemophagocytic syndrome (EBV-AHS), a disease caused by a

267 ld white boy diagnosed with virus-associated hemophagocytic syndrome (VAHS).

268 Histiocytes in Rosai-Dorfman disease and hemophagocytic syndrome also coexpressed CCR6 and CCR7,

269 , several causes of prolonged fever (such as hemophagocytic syndrome and atypical cat scratch disease

270 Most patients with GS display the hemophagocytic syndrome and have mutations in RAB27A, wh

271 ble neurological involvement, along with the hemophagocytic syndrome and immunologic abnormalities.

272 Multicentric Castleman disease and hemophagocytic syndrome are other potential HHV-8-induce

273 Hemophagocytic syndrome diagnosis was standardized and b

274 ocytophilum causes macrophage activation and hemophagocytic syndrome features.

275 We report a severe case of babesiosis and hemophagocytic syndrome in an asplenic renal transplant

276 iteria to be highly discriminant identifying hemophagocytic syndrome in critically ill patients.

277 lymphohistiocytosis criteria was 4 (4-5) in hemophagocytic syndrome+ and 1 (0-1) in hemophagocytic s

278 Median HScore was 235 (205-262) in hemophagocytic syndrome+ and 42 (18-62) in hemophagocyti

279 x 2), the median HScore was 234 (205-262) in hemophagocytic syndrome+ patients versus 49 (18-71) in h

280 eral conditions share some similarities with hemophagocytic syndrome, explaining the poor predictive

281 ted; however, 1 patient at day +13 developed hemophagocytic syndrome, followed by graft rejection at

282 Hospital mortality was 45.8% in hemophagocytic syndrome- patients (n = 66) and 38.8% in

283 ytic syndrome+ patients versus 49 (18-71) in hemophagocytic syndrome- patients (p < 0.001).

284 ) in hemophagocytic syndrome+ and 1 (0-1) in hemophagocytic syndrome- patients (p < 0.001).

285 n hemophagocytic syndrome+ and 42 (18-62) in hemophagocytic syndrome- patients (p < 0.001); number of

286 Control group (hemophagocytic syndrome-) consisted in patients included

287 nia, and erythrophagocytosis indicative of a hemophagocytic syndrome.

288 is aggressive and frequently associated with hemophagocytic syndrome.

289 dependent experts ruled out the diagnosis of hemophagocytic syndrome.

290 ity hospital which is a reference center for hemophagocytic syndrome.

291 man disease with microlymphomas and a severe hemophagocytic syndrome.

292 h are primary pathological manifestations of hemophagocytic syndrome/hemophagocytic lymphohistiocytos

293 ecific diagnostic criteria for the different hemophagocytic syndromes are being developed.

294 New work in the field suggests that not all hemophagocytic syndromes are equal.

295 despite clinical similarities the different hemophagocytic syndromes are indeed likely heterogeneous

296 t at least two different mechanisms by which hemophagocytic syndromes arise: enhanced antigen present

297 to the diagnosis, etiology, and treatment of hemophagocytic syndromes including MAS.

298 of a larger group of diseases referred to as hemophagocytic syndromes that are seen in infections, ma

299 eripheral cytopenias, associated or not with hemophagocytic syndromes, and to acute hepatitis syndrom

300 rent treatment strategies for the variety of hemophagocytic syndromes.