ライフサイエンスコーパス: HLH

1 HLH is associated with mutations in lymphocyte cytolytic

2 HLH occurs as both acquired and familial hemophagocytic

3 HLH-1 binding did not predict proximate regulatory actio

4 HLH-30 also upregulates MML-1 upon germline removal.

5 HLH-30 was activated shortly after Staphylococcus aureus

6 CEH-30, EGL-27, EGL-5, ELT-3, EOR-1, GEI-11, HLH-1, LIN-11, LIN-13, LIN-15B, LIN-39, MAB-5, MDL-1, ME

7 bditis elegans E-protein helix-loop-helix-2 (HLH-2) functions as a homodimer in directing development

8 on of 1) mesodermal intrinsic factors MAB-5, HLH-1 and FOZI-1, 2) differential POP-1 (TCF/LEF) transc

9 occurrence of systemic reactivations (>/=5/8 HLH criteria), partial systemic flares (<5 criteria and

10 uronal differentiation and identified ID1, a HLH inhibitor of premature neurogenesis, as a target.

11 malignant, or metabolic conditions (acquired HLH).

12 ient mice still developed the aforementioned HLH-like symptoms.

13 Although HLH is well described in the pediatric population, less

14 hat repeated stimulation of TLR9 produced an HLH/MAS-like syndrome on a normal genetic background, wi

15 ir associated transacting factors (PAL-1 and HLH-1) that are crucial for the temporal-spatial express

16 encode the proneural bHLH proteins NGN-1 and HLH-2 and the Otx homeodomain protein CEH-36.

17 Ectopic expression of HLH-2 and HLH-3 together promoted expression of neuronal features

18 MML-1/MXL-2 and HLH-30 transcriptomes show both shared and preferential

19 a), partial systemic flares (<5 criteria and HLH-directed treatment), isolated central nervous system

20 onfirms the presence of hemophagocytosis and HLH pathology.

21 ntiation between secondary post-HSCT HLH and HLH related to the genetic defect difficult.

22 estimated incidence rates for malignancy and HLH as events/1000 patient-years of follow-up.

23 unadjusted incidence rates of malignancy and HLH in pediatric patients with IBD exposed to infliximab

24 Murine models of MAS and HLH illustrate that interferon-gamma (IFNgamma) is the d

25 Preparative regimen and HLH activity affected outcomes, and of RIC patients repo

26 without exposure to Epstein-Barr virus, and HLH is commonly recurrent in these patients.

27 by interacting antagonistically with another HLH factor, IBH1.

28 hat disease severity as determined by age at HLH onset differed significantly, with a severity gradie

29 g hypocotyl in far-red 1 (HFR1), an atypical HLH protein.

30 he precocious induction of myogenic basic (b)HLH proteins, the activity of myogenic bHLH proteins, or

31 ion of DNA binding activities of B-ZIP and B-HLH-ZIP transcription factors.

32 pends in part on the function of the Mash1 b-HLH transcription factor.

33 two basic helix-loop-helix leucine zipper (B-HLH-ZIP) [USF (upstream stimulating factor) and Mitf] pr

34 roteins to interact with the canonical basic HLH clock proteins BMAL1 and CLOCK.

35 protein that inhibits the function of basic HLH E protein transcription factors in lymphoid cells, h

36 riven down-regulation can target other basic HLH (bHLH) dimers as well.

37 ID3, decreased levels of the proneural basic HLH (bHLH) transcriptional regulators TCF4 and NEUROD6 a

38 insights suggest significant overlap between HLH and severe inflammation in a variety of clinical con

39 f1(-/-) and Rab27a(-/-) mice with full-blown HLH syndrome were treated with a clinically relevant dos

40 Clinical manifestations of both HLH subtypes are often precipitated by a viral infection

41 We also mapped global factor occupancy by HLH-1, and created a genetic interaction map that identi

42 enes that are transcriptionally regulated by HLH-2: the protocadherin cdh-3, and two genes encoding s

43 neral feature of developmental regulation by HLH proteins.

44 To date, XLP2 has been found to cause HLH with and without exposure to Epstein-Barr virus, and

45 ce from the fatal outcome that characterizes HLH-like disease and was also sufficient to reduce HLH-l

46 of macrophage overactivation characterizing HLH in 2 murine models.

47 Clinically, HLH is defined by severe inflammation and potentially fa

48 should be considered for addition to current HLH criteria.

49 The C. elegans homolog of E/Daughterless, HLH-2, was previously implicated in hDTC specification.

50 reclinical studies have increasingly defined HLH as a syndrome of abnormal and excessive T-cell activ

51 nce alignment and variant filtering detected HLH gene mutations and potential disease-causing variant

52 mab treatment and observed that he developed HLH.

53 Nine of 10 patients developed HLH by 8 years of age.

54 ancy and all 5 of the patients who developed HLH had been exposed to thiopurines; 10 patients with ma

55 logic test results in patients who developed HLH in adulthood.

56 a single patient was reported who developed HLH.

57 s did indeed increase the risk of developing HLH immunopathology after lymphocytic choriomeningitis v

58 f bHLH-encoding genes-and therefore distinct HLH-2:bHLH dimers-and formulate a "bHLH code" hypothesis

59 gest that increased T-cell activation drives HLH, potential abnormalities of T-cell activation have n

60 othesized that immune hyperactivation during HLH is caused by heightened infection, defective apoptos

61 s can functionally substitute for C. elegans HLH-2 in regulating AC development and also display dime

62 f possible, efforts should be made to ensure HLH remission before HCT in these patients.

63 ology but also is effective against existing HLH, cytotoxicity-impaired Prf1(-/-) and Rab27a(-/-) mic

64 observed in untreated patients experiencing HLH flares.

65 ID2 is a rhythmically expressed HLH transcriptional repressor.

66 milial hemophagocytic lymphohistiocytosis (F-HLH) and Griscelli syndrome type 2 (GS) are life-threate

67 (GS) and PRF1, UNC13D, STX11, and STXBP2 (F-HLH).

68 by the Zic factor REF-2 and the bHLH factor HLH-2 directly activates ttx-3 expression in the AIY mot

69 Here we identify the transcription factor HLH-2, the C. elegans ortholog of Drosophila Daughterles

70 helix-loop-helix (bHLH) transcription factor HLH-2, the sole ortholog of the E proteins mammalian E2A

71 basic helix-loop-helix transcription factor, HLH-30.

72 Familial HLH is well recognized in children but rarely diagnosed

73 tions affecting cytotoxic function (familial HLH) or be secondary to infectious, rheumatologic, malig

74 derance of hypomorphic mutations in familial HLH-causing genes correlates with the later-onset clinic

75 We conclude that late-onset familial HLH occurs more commonly than was suspected previously.

76 converted nonfatal disease course into fatal HLH, identifying T-cell exhaustion as an important facto

77 nemia, and a cytokine profile diagnostic for HLH.

78 luding blinatumomab, should be monitored for HLH, and cytokine-directed therapy may be considered in

79 st performance in 1614 patients referred for HLH evaluation.

80 g heterozygous RAB27A mutation, the risk for HLH in HPS2 remains unclear.

81 HPS2 confers a risk for HLH that is lower than in Griscelli or Chediak-Higashi s

82 our results demonstrate a conserved role for HLH-30 and TFEB in autophagy, and possibly longevity, an

83 ariety of conditions and is not specific for HLH in adults.

84 on, marked hyperferritinemia is specific for HLH.

85 omerase II, is the mainstay of treatment for HLH, although its therapeutic mechanism remains unknown.

86 r the wide disease spectrum that ranges from HLH secondary to severe infection to FHL.

87 iption 1 activation and led to recovery from HLH manifestations in both murine models.

88 IC patients reported to be in remission from HLH, survival is 86% (P = .03).

89 totoxicity testing for screening for genetic HLH and should be considered for addition to current HLH

90 ein, occurring through the Helix-Loop-Helix (HLH) and PER-ARNT-SIM (PAS) domains, is needed to conver

91 FIGLA binding to the TCF3 helix-loop-helix (HLH) domain.

92 zol-resistant (PRE) family helix-loop-helix (HLH) factors, which promote cell elongation by interacti

93 vergent non-Ca(2+)-binding helix-loop-helix (HLH) motif packed against a canonical Ca(2+)-binding EF-

94 We demonstrate that the helix-loop-helix (HLH) protein Extramacrochaetae (Emc) regulates Ci(155) l

95 Id1, a helix-loop-helix (HLH) protein that inhibits the function of basic HLH E p

96 We demonstrate that the helix-loop-helix (HLH) protein, HEB, directly associates with the Polycomb

97 targets are the inhibitory helix-loop-helix (HLH) proteins inhibitor of DNA binding (Id) 2 and Id3, b

98 Helix-loop-helix (HLH) proteins play a profound role in the process of dev

99 unction of the Id3 and E2A helix-loop-helix (HLH) proteins.

100 d an important role of the helix-loop-helix (HLH) transcription factor Ascl1 in early generation of O

101 tae (emc), which encodes a helix-loop-helix (HLH) transcription factor.

102 , we identify a network of helix-loop-helix (HLH) transcriptional regulators controlled by MYT1L, as

103 ing genes (Id1-Id4) encode helix-loop-helix (HLH) transcriptional repressors associated with developm

104 ion of a common downstream helix-loop-helix (HLH)/bHLH network, thus forming an incoherent feed-forwa

105 A conserved helix-turn-helix (HLH) that is part of this site interacts with the portal

106 dy, 103 patients transplanted for hereditary HLH (2000-2013) and DC permanently or transiently <75% (

107 te HIF1 target gene promoters in a HIF1alpha HLH/PAS and N-TAD dependent manner while HIF2alpha inter

108 Physically, HIF1alpha HLH and PAS domains are required for its interaction wit

109 clinical observations are also changing how HLH is conceptualized.

110 differentiation between secondary post-HSCT HLH and HLH related to the genetic defect difficult.

111 Deletion of the ID2 HLH domain rendered ID2 ineffective at inhibiting CLOCK-

112 From this screen, we identified HLH-3, the C. elegans homolog of a mammalian proneural p

113 This work identifies HLH-2 as a regulator of the invasive phenotype in the AC

114 ophagy, and possibly longevity, and identify HLH-30 as a uniquely important transcription factor for

115 be associated with heterozygous mutations in HLH genes.

116 ubly or triply heterozygous for mutations in HLH-associated genes, those coding for perforin, Rab27a,

117 kine-driven hyperinflammation that occurs in HLH.

118 f cytokines, including those overproduced in HLH.

119 tor of myeloid and lymphoid proliferation in HLH, and suggest that blockade of this signaling molecul

120 tor for determination of disease severity in HLH.

121 le-specific transcription factors, including HLH-1/MRF and UNC-120/SRF, which together orchestrate sp

122 he characteristics of LCMV infection-induced HLH in the murine counterparts of the 3 human conditions

123 ibition using a model of primary (inherited) HLH in which perforin-deficient (Prf1(--)) mice are infe

124 udies reveal how an extensive interdependent HLH transcription factor network distributes responsibil

125 a cell non-autonomous role for the intrinsic HLH factor, Id2a, in regulating retinoblast proliferatio

126 referential outputs including MDL-1/MAD-like HLH factor required for longevity.

127 Here we identify a Myc-like HLH transcription factor network comprised of Mondo/Max-

128 ial role of NK-cell cytotoxicity in limiting HLH-like immunopathology, highlighting the important rol

129 ptoms of hemophagocytic lymphohistiocytosis (HLH) after infection with lymphocytic choriomeningitis v

130 disorder hemophagocytic lymphohistiocytosis (HLH) after infection.

131 dence of hemophagocytic lymphohistiocytosis (HLH) and a lack of lymphoma, suggesting that classificat

132 Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are 2 simi

133 romes of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are both c

134 istic of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS).

135 SCT) for hemophagocytic lymphohistiocytosis (HLH) at the cost of more frequent mixed chimerism.

136 Primary hemophagocytic lymphohistiocytosis (HLH) can be caused by biallelic mutations in PRF1, encod

137 Hemophagocytic lymphohistiocytosis (HLH) comprises an emerging spectrum of inherited and non

138 Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune ac

139 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disease.

140 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immunological disorder that i

141 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an uncontro

142 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome that often occurs in

143 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, characterized by se

144 Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, heterogeneous, hyperinflammm

145 Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory disorder with a poor prognos

146 Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease affecting mostly

147 Familial hemophagocytic lymphohistiocytosis (HLH) is a rare primary immunodeficiency disorder charact

148 Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of uncontrolled immune activatio

149 Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory condition driven by excess

150 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occu

151 Hemophagocytic lymphohistiocytosis (HLH) is an immune dysregulatory syndrome that is associa

152 Hemophagocytic lymphohistiocytosis (HLH) is an inborn disorder of immune regulation caused b

153 forms of hemophagocytic lymphohistiocytosis (HLH) is crucial for treatment decisions.

154 primary hemophagocytic lymphohistiocytosis (HLH) occurs in infancy resulting from homozygous mutatio

155 ng fatal hemophagocytic lymphohistiocytosis (HLH) triggered predominantly by Epstein-Barr virus, lymp

156 nts with hemophagocytic lymphohistiocytosis (HLH) undergoing allogeneic hematopoietic cell transplant

157 arity to hemophagocytic lymphohistiocytosis (HLH), and some authors prefer the term secondary HLH to

158 odels of hemophagocytic lymphohistiocytosis (HLH), and the HLH-sibling macrophage activation syndrome

159 disorder hemophagocytic lymphohistiocytosis (HLH), characterized by uncontrolled CD8 T-cell and macro

160 milar to hemophagocytic lymphohistiocytosis (HLH), which is caused by viral infection of a host with

161 underlie hemophagocytic lymphohistiocytosis (HLH), which requires hematopoietic cell transplantation

162 oallelic hemophagocytic lymphohistiocytosis (HLH)-associated mutations affecting codon 65 of STXPB2,

163 ients to hemophagocytic lymphohistiocytosis (HLH).

164 ading to hemophagocytic lymphohistiocytosis (HLH).

165 familial hemophagocytic lymphohistiocytosis (HLH).

166 r called hemophagocytic lymphohistiocytosis (HLH).

167 reactive hemophagocytic lymphohistiocytosis (HLH).

168 ncluding hemophagocytic lymphohistiocytosis (HLH).

169 bility to hemophagocytic lymphohistocytosis (HLH).

170 (H1N1) infection met 44% and 81% of modified HLH-2004 and MAS criteria, respectively.

171 d laboratory features of HLH, using modified HLH-2004 and macrophage activation syndrome (MAS) criter

172 carry a monoallelic mutation in one or more HLH-associated genes.

173 stantially alleviated all symptoms of murine HLH and allowed prolonged survival.

174 The c-Myc HLH-bZIP protein has been implicated in physiological or

175 18) are BWM-specific and enriched for nearby HLH-1 binding.

176 is virus (LCMV) and secondary (noninherited) HLH in which C57BL/6 mice receive repeated injections of

177 gulates nuclear localization and activity of HLH-30/TFEB, a convergent regulator of autophagy, lysoso

178 Our structure-function analysis of HLH-2 indicates that dimerization drives its degradation

179 We report the first case of HLH due to Bartonella henselae infection in a patient wi

180 In a cohort of HLH patients with genetic abnormalities expected to resu

181 ough cytotoxic T lymphocyte (CTL) control of HLH development is well documented, the role for natural

182 tion and protects against the development of HLH and potentially other immunopathological conditions.

183 oxicity can contribute to the development of HLH, we generated mice that were doubly or triply hetero

184 The diagnosis of HLH is based on a constellation of clinical and laborato

185 e 1531 patients with a clinical diagnosis of HLH; 175 patients were 18 years or older.

186 Ectopic expression of HLH-2 and HLH-3 together promoted expression of neuronal

187 displayed the clinicopathologic features of HLH, as set forth in the Histiocyte Society diagnostic g

188 (LCMV) present typical clinical features of HLH, including splenomegaly, elevated serum IFNgamma, an

189 us, pearl mice developed all key features of HLH, linked to impaired virus control caused by a modera

190 yzed for clinical and laboratory features of HLH, using modified HLH-2004 and macrophage activation s

191 atients with a so-called "secondary" form of HLH, which develops in the aftermath of infection, autoi

192 Familial forms of HLH (FHL), which are increasingly found also in adolesce

193 Inherited forms of HLH are caused by biallelic mutations in several effecto

194 Acquired forms of HLH are encountered in association with (usually) viral

195 Familial forms of HLH have been attributed to mutations in the genes encod

196 rimary, and perhaps even secondary, forms of HLH.

197 ECATE (HEC) proteins, another small group of HLH proteins, antagonistically regulate PIFs to promote

198 Nuclear localization of HLH-30 is increased in all six Caenorhabditis elegans mo

199 faithfully reproduced the manifestations of HLH after lymphocytic choriomeningitis virus (LCMV) infe

200 he clinical and laboratory manifestations of HLH, including weight loss, organomegaly, anemia, thromb

201 We used a murine model of HLH to examine how perforin controls immune activation,

202 We used a murine model of HLH, involving lymphocytic choriomeningitis virus infect

203 t of mixed chimerism using a murine model of HLH.

204 Animal models of HLH suggest that disease is driven by IFN-gamma produced

205 ural killer (NK) cell function, mutations of HLH-associated genes in patients with systemic JIA have

206 of the JAK1/2 not only prevents the onset of HLH immunopathology but also is effective against existi

207 inct longevity models, and overexpression of HLH-30 extends lifespan.

208 al role for cytokines in the pathogenesis of HLH, we sought to examine whether the inhibition of JAK

209 the clinical manifestations and patterns of HLH and describe our approach to the diagnosis and thera

210 Moreover, the high percentage of HLH gene mutations suggests they are risk factors for mo

211 h represent a model for FHL2, progression of HLH was not fatal.

212 may be challenging because of the rarity of HLH, its variable presentation, and the time required to

213 Early recognition of HLH and B. henselae through liver biopsy and serological

214 Increased recognition of HLH in older children and adults, sometimes in associati

215 e expanding understanding and recognition of HLH, driving an evolution in how it is defined, and sugg

216 Reduction of HLH-2 function by RNAi or with a hypomorphic allele caus

217 Further, we show that reduction of HLH-2 function causes defects in polarization of F-actin

218 on to hematopoiesis and prevented relapse of HLH in all but 1 patient.

219 merism and uncertainty regarding the risk of HLH recurrence.

220 tical to prevent the catastrophic sequela of HLH.

221 milar gradient in the phenotypic severity of HLH manifestations.

222 Strikingly, the severity of HLH was not correlated with the LCMV load and not fully

223 t mice displayed a more complete spectrum of HLH, including splenomegaly, coagulopathy, and decreased

224 he same enhancer element is also a target of HLH-1 positive auto regulation, underlying (at least in

225 characteristics, diagnosis, and treatment of HLH in adults.

226 Treatment of HLH includes immune-suppressive and immune-modulatory ag

227 xperience gained through treatment trials of HLH and MAS in childhood may inform study design for the

228 ions, is challenging the traditional view of HLH as either a distinctly familial or a sporadic disord

229 he critical protective effect of NK cells on HLH was independent of interferon-gamma secretion and ch

230 sociate with increased risk of malignancy or HLH in pediatric patients with IBD.

231 t event for the development of malignancy or HLH in pediatric patients with IBD.

232 k of malignancy (0.46/1000 patient-years) or HLH (0.0/1000 patient-years) in patients exposed to IFX

233 e we show that the predicted TFEB orthologue HLH-30 regulates autophagy in Caenorhabditis elegans and

234 of donor chimerism (DC) required to prevent HLH reactivation in humans remains to be determined.

235 Like previous HLH models, TLR9-induced MAS was IFN-gamma dependent; ho

236 Primary HLH is caused by mutations in granule-mediated cytotoxic

237 ed to its central pathogenic role in primary HLH.

238 key pathogenic cytokine in models of primary HLH, were the highest.

239 t dissimilarity from mouse models of primary HLH.

240 In these models and patients with "primary" HLH, the antigen persists due to genetic defects, result

241 ts together with a second proneural protein, HLH-2, and in parallel to HLH-3 to promote I4 neurogenes

242 A recombinant HLH peptide competes with gp17 for portal binding and bl

243 patients presented in infancy, and recurrent HLH was common.

244 ke disease and was also sufficient to reduce HLH-like manifestations.

245 ly, PIF4 bound all direct and down-regulated HLH/bHLH targets of IBH1 and IBL1.

246 ed in terms of the severity of the resulting HLH manifestations.

247 Secondary HLH presents after infancy and may be associated with he

248 ic distinction between primary and secondary HLH by contributing to HLH via a partial dominant-negati

249 e pathogenesis of virus-associated secondary HLH as opposed to its central pathogenic role in primary

250 n animal model of virus-associated secondary HLH by infecting immunocompetent wild-type mice with the

251 ients with a clinical diagnosis of secondary HLH, 13 of 59 (22%) had abnormal resting NK-cell degranu

252 lygenic model of the occurrence of secondary HLH.

253 , and some authors prefer the term secondary HLH to describe it.

254 ule-mediated cytotoxicity, whereas secondary HLH occurs, without a known genetic background, in a con

255 6%) patients classified as having "sporadic" HLH, 43 had monoallelic mutations in one of the FHL-defi

256 tients referred for evaluation for suspected HLH.

257 We suggest that the clinical syndrome HLH generally results from the combined effects of an ex

258 f hemophagocytic lymphohistiocytic syndrome (HLH).

259 We conclude that HLH-2 functions in both sexes to promote leader cell spe

260 l Caenorhabditis elegans, we discovered that HLH-30 (known as TFEB in mammals) is a key transcription

261 We also find that HLH-2 functions in males to direct linker cell specifica

262 Genetic analysis indicates that HLH-2 has functions outside of the FOS-1A pathway.

263 Here we report that HLH-2 is also critical for hDTC maintenance, hDTC niche

264 Although this observation suggests that HLH may have a polygenic mode of inheritance, the latter

265 The HLH continued to progress after discontinuation of blina

266 The HLH phenotype was further characterized by hyperactive C

267 The HLH protein Extramacrocheate (Emc), which forms heterodi

268 Among the HLH proteins expressed in differentiating erythroid cell

269 TOH1 is closely correlated with HEYL and the HLH inhibitory transcription factors ID1, ID2, and ID4.

270 hagocytic lymphohistiocytosis (HLH), and the HLH-sibling macrophage activation syndrome (MAS).

271 CD8(+) T cells could not inhibit or cure the HLH-like syndrome, highlighting a first dissimilarity fr

272 mples were collected and analyzed during the HLH episode.

273 nt shows that Notch signaling depends on the HLH protein Extramacrochaetae.

274 kk1 kinase activity but does not require the HLH domain.

275 interaction between the proteins is via the HLH region.

276 All of these HLH proteins exhibit distinct functions during the diffe

277 This HLH motif may have evolved from a canonical EF-hand foun

278 Lymphocyte defects thought to contribute to HLH development in SLAM-Associated Protein deficiency we

279 mpair lytic granule fusion and contribute to HLH.

280 primary and secondary HLH by contributing to HLH via a partial dominant-negative effect.

281 cellular and molecular mechanisms leading to HLH in Unc13d(jinx/jinx) mice, in which cytolytic functi

282 proneural protein, HLH-2, and in parallel to HLH-3 to promote I4 neurogenesis.

283 contribution of these lymphocyte subsets to HLH-like disease severity after lymphocytic choriomening

284 We analyzed susceptibility to HLH in the pearl mouse model of HPS2.

285 ent with HLH and 2 with incomplete transient HLH-like episodes, although cytotoxicity or degranulatio

286 vity and mechanism of etoposide for treating HLH and found that it substantially alleviated all sympt

287 Thus, etoposide treats HLH by selectively eliminating pathologic, activated T c

288 In addition, a tripartite HLH/bHLH module feedback regulates PIFs and additional b

289 tions lead to a hypothesis in which a unique HLH-portal interaction in the symmetrically mismatched c

290 While HLH is either inherited or acquired, children with sever

291 ffering a partial explanation for widespread HLH-1 occupancy.

292 we identified only 1 additional patient with HLH and 2 with incomplete transient HLH-like episodes, a

293 in patients with MAS, and some patients with HLH or MAS lack defects in cytotoxic T cell killing.

294 cantly improves the outcome of patients with HLH undergoing allogeneic HCT.

295 Forty patients with HLH underwent allogeneic HCT between 2003-2009 at Cincin

296 to persistent inflammation in patients with HLH.

297 yzed a total of 500 unselected patients with HLH.

298 e-associated protein (SAP), may present with HLH.

299 We report two unrelated teenagers with HLH and an identical heterozygous RAB27A mutation (c.259

300 logics (malignancy: 1.12/1000 patient-years; HLH: 0.56/1000 patient-years).