ライフサイエンスコーパス: hemophagocytic

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

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

3 vation syndrome and other clinically similar hemophagocytic disorders.

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

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

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

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

8 Hemophagocytic lymphohistiocytoses represent a rare but

9 e molecular and pathophysiologic features of hemophagocytic lymphohistiocytoses.

10 of patients with both primary and secondary hemophagocytic lymphohistiocytoses.

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

12 Familial hemophagocytic lymphohistiocytosis (F-HLH) and Griscelli

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

14 Familial hemophagocytic lymphohistiocytosis (FHL) is a geneticall

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

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

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

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

19 Familial hemophagocytic lymphohistiocytosis (FHL) is an inherited

20 Familial hemophagocytic lymphohistiocytosis (FHL) is caused by ge

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

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

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

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

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

26 -4, in which mutations cause type 3 familial hemophagocytic lymphohistiocytosis (FHL3), a fatal disea

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

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

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

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

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

32 Hemophagocytic lymphohistiocytosis (HLH) and macrophage

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

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

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

36 Primary hemophagocytic lymphohistiocytosis (HLH) can be caused b

37 Hemophagocytic lymphohistiocytosis (HLH) comprises an em

38 Hemophagocytic lymphohistiocytosis (HLH) is a devastatin

39 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

40 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

41 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

42 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

43 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

44 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

45 Hemophagocytic lymphohistiocytosis (HLH) is a life-threa

46 Hemophagocytic lymphohistiocytosis (HLH) is a rare disor

47 Hemophagocytic lymphohistiocytosis (HLH) is a rare disor

48 Hemophagocytic lymphohistiocytosis (HLH) is a rare infla

49 Hemophagocytic lymphohistiocytosis (HLH) is a rare life-

50 Familial hemophagocytic lymphohistiocytosis (HLH) is a rare prima

51 Hemophagocytic lymphohistiocytosis (HLH) is a rare syndr

52 Hemophagocytic lymphohistiocytosis (HLH) is a severe inf

53 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome o

54 Hemophagocytic lymphohistiocytosis (HLH) is an immune dy

55 Hemophagocytic lymphohistiocytosis (HLH) is an inborn di

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

89 The better understood familial hemophagocytic lymphohistiocytosis is a constellation of

90 Hemophagocytic lymphohistiocytosis is a disease of abnor

91 Hemophagocytic lymphohistiocytosis is a hyperinflammator

92 Hemophagocytic lymphohistiocytosis is a life-threatening

93 The hemophagocytic lymphohistiocytosis is probably underdiag

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

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

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

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

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

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

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

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

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

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

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

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

106 Familial hemophagocytic lymphohistiocytosis type 5 (FHL5) is caus

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

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

109 A liver biopsy showed hemophagocytic lymphohistiocytosis with exuberant infilt

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

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

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

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

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

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

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

117 ncluding fulminant infectious mononucleosis, hemophagocytic lymphohistiocytosis, lymphoproliferative

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

119 In familial hemophagocytic lymphohistiocytosis, Prf-deficient infant

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

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

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

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

124 escribed by examining patients with familial hemophagocytic lymphohistiocytosis.

125 ppropriately classified as X-linked familial hemophagocytic lymphohistiocytosis.

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

127 g them are Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis.

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

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

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

131 with familial and virus-associated reactive hemophagocytic lymphohistiocytosis.

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

133 ked lymphoproliferative disease and familial hemophagocytic lymphohistiocytosis.

134 erest in discussing a specific treatment for hemophagocytic lymphohistiocytosis.

135 rimary immunodeficiencies including familial hemophagocytic lymphohistiocytosis.

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

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

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

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

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

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

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

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

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

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

146 ctivation and expansion of T lymphocytes and hemophagocytic macrophages.

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

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

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

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

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

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

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

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

155 ocytophilum causes macrophage activation and hemophagocytic syndrome features.

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

157 man disease with microlymphomas and a severe hemophagocytic syndrome.

158 nia, and erythrophagocytosis indicative of a hemophagocytic syndrome.

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

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

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

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

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

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

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

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

167 rent treatment strategies for the variety of hemophagocytic syndromes.