ライフサイエンスコーパス: aplastic

1 c stem cell transplantation (HSCT) in severe aplastic anaemia (SAA) have improved steadily over the p

2 the placebo-controlled period: one event of aplastic anaemia and five serious adverse events related

3 ns have severe disease, with most developing aplastic anaemia by the age of 10 years.

4 Acquired aplastic anaemia can be effectively treated by allogenei

5 Similarly to other autoimmune diseases, aplastic anaemia has a varied clinical course; some pati

6 Aplastic anaemia in adults is usually acquired, but rare

7 Aplastic anaemia is a bone-marrow-failure syndrome chara

8 Aplastic anaemia is a rare haemopoietic stem-cell disord

9 Aplastic anaemia is caused by an intrinsic defect of hae

10 The pathophysiology of acquired aplastic anaemia is immune mediated in most cases; autor

11 The use of alternative donor sources for aplastic anaemia patients remains limited and problemati

12 The main cause of aplastic anaemia remains elusive.

13 rrow transplantation in patients with severe aplastic anaemia remains to be established.

14 To test the hypothesis that aplastic anaemia results from antigen-specific lymphocyt

15 The CDR3 sequence repertoire in aplastic anaemia showed much redundancy compared with he

16 Blood samples from 54 patients with aplastic anaemia were subjected to flow cytometry to def

17 ponse (no longer meeting criteria for severe aplastic anaemia).

18 udy, patients (aged </=65 years) with severe aplastic anaemia, adequate organ function, and an unrela

19 ia, three of 27 patients with constitutional aplastic anaemia, but in none of 214 normal controls (p<

20 order associated with chromosomal fragility, aplastic anaemia, congenital abnormalities and a high ri

21 rited disorder associated with a progressive aplastic anaemia, diverse congenital abnormalities and c

22 tic syndrome commonly arise in patients with aplastic anaemia, showing a pathophysiological link betw

23 indicate that, in a subset of patients with aplastic anaemia, the disorder might be associated with

24 ations in two of 17 patients with idiopathic aplastic anaemia, three of 27 patients with constitution

25 onse can be identified in many patients with aplastic anaemia, which is evidence for an underlying an

26 unosuppressive agent for treatment of severe aplastic anaemia, with a response rate similar to that w

27 not develop leukaemia spontaneously develop aplastic anaemia, with the concomitant accumulation of d

28 c" immune response in the pathophysiology of aplastic anaemia.

29 ormal adults, and can masquerade as acquired aplastic anaemia.

30 nita--an inherited syndrome characterised by aplastic anaemia.

31 r unrelated donor transplantation for severe aplastic anaemia.

32 r unrelated donor transplantation for severe aplastic anaemia.

33 ematopoietic stem cell pool confers profound aplastic anaemia.

34 ndromes including dyskeratosis congenita and aplastic anaemia.

35 tumors (3), leukemia (3), lymphoma (1), and aplastic anemia (1).

36 The distinction between acquired aplastic anemia (AA) and hypocellular myelodysplastic sy

37 Some (~ 3%) sporadic aplastic anemia (AA) and idiopathic pulmonary fibrosis c

38 Aplastic anemia (AA) and myelodysplasia (MDS) are forms

39 nisms are involved in the pathophysiology of aplastic anemia (AA) and myelodysplastic syndrome (MDS).

40 cytopenias and can cooccur in the context of aplastic anemia (AA) and myelodysplastic syndromes (MDS)

41 patients with previously untreated nonsevere aplastic anemia (AA) as defined by a neutrophil count of

42 ain (VB) T-cell receptor (TCR) repertoire in aplastic anemia (AA) at initial presentation and after i

43 in 4 of 91 patients with apparently acquired aplastic anemia (AA) but not in 276 ethnically matched c

44 We studied the role of Th17 cells in aplastic anemia (AA) by isolating Th17 cells from patien

45 Immune mediation of aplastic anemia (AA) has been inferred from clinical res

46 An immune pathophysiology for acquired aplastic anemia (AA) has been inferred from the responsi

47 Improved survival in aplastic anemia (AA) has shown a high incidence of late

48 Severe aplastic anemia (AA) is a bone marrow (BM) failure (BMF)

49 Aplastic anemia (AA) is a disease characterized by T-cel

50 accumulating evidence strongly suggests that aplastic anemia (AA) is a T cell-mediated autoimmune dis

51 Idiopathic aplastic anemia (AA) is an immune-mediated and serious f

52 Aplastic anemia (AA) is characterized by hypocellular ma

53 Refractory aplastic anemia (AA) is defined as a lack of response to

54 A serious complication of aplastic anemia (AA) is its evolution to clonal hematolo

55 ole of CD4(+) T cells in the pathogenesis of aplastic anemia (AA) is not well characterized.

56 We have hypothesized that in aplastic anemia (AA) the presence of antigen-specific T

57 teristics and outcome of posttransplantation aplastic anemia (AA) were determined in 12 of 1,736 pati

58 Aplastic anemia (AA), a potentially fatal disease, may b

59 in 55, 34, 43, and 5 patients with acquired aplastic anemia (AA), autoimmune uveitis, systemic lupus

60 In aplastic anemia (AA), contraction of the stem cell pool

61 in bone marrow failure syndromes, including aplastic anemia (AA), paroxysmal nocturnal hemoglobinuri

62 samples from a large number of patients with aplastic anemia (AA), paroxysmal nocturnal hemoglobinuri

63 patients with dyskeratosis congenita (DC) or aplastic anemia (AA).

64 ematologic improvement in most patients with aplastic anemia (AA).

65 its serologic split HLA-DR15 in both MDS and aplastic anemia (AA).

66 ch a relationship also has been reported for aplastic anemia (AA).

67 th suppressed hematopoiesis, including frank aplastic anemia (AA).

68 of patients with congenital neutropenia and aplastic anemia (AA).

69 Hepatitis-associated aplastic anemia (HAA) is a syndrome of bone marrow failu

70 inant hepatitis (FH) or hepatitis-associated aplastic anemia (HAA).

71 lose relationship between PNH and idiopathic aplastic anemia (IAA), it has been suggested that the 2

72 We treated 17 patients with moderate aplastic anemia (mAA) with 1 mg/kg every 2 weeks for 3 m

73 Severe aplastic anemia (SAA) appears to be an immunologically m

74 Severe aplastic anemia (SAA) can be successfully treated with a

75 bone marrow (BM) transplantation for severe aplastic anemia (SAA) has improved, with survival rates

76 Survival in severe aplastic anemia (SAA) has markedly improved in the past

77 Severe aplastic anemia (SAA) is a life-threatening bone marrow

78 Severe aplastic anemia (SAA) is a life-threatening bone marrow

79 Acquired severe aplastic anemia (SAA) is a rare hematologic disease asso

80 First-line therapy of severe aplastic anemia (SAA) with high-dose cyclophosphamide ca

81 In contrast to severe aplastic anemia (sAA), the appropriate management of pat

82 bone marrow transplantation (BMT) for severe aplastic anemia (SAA).

83 been promoted as curative therapy for severe aplastic anemia (SAA).

84 fective in restoring hematopoiesis in severe aplastic anemia (SAA).

85 itioning regimen in HSCT for acquired severe aplastic anemia (SAA).

86 About one-third of patients with acquired aplastic anemia also have short telomeres, which in some

87 s from 124 patients with apparently acquired aplastic anemia and 282 control subjects for sequence va

88 durable treatment-free remissions in severe aplastic anemia and a variety of other autoimmune disord

89 ightened cellular sensitivity to DNA damage, aplastic anemia and cancer susceptibility.

90 activation in the bone marrow occurs such as aplastic anemia and certain infectious conditions.

91 ies had shown that LCMV infection results in aplastic anemia and death in most of these mice and that

92 In FA-C, there was a later age of onset of aplastic anemia and fewer somatic abnormalities in patie

93 the diagnosis and treatment of patients with aplastic anemia and highlight a role for the THPO-MPL pa

94 omerase, cause short telomeres in congenital aplastic anemia and in some cases of apparently acquired

95 , remains controversial for the treatment of aplastic anemia and inherited bone marrow failure syndro

96 Hematologic disorders such as aplastic anemia and leukemia induced by chloramphenicol

97 mphomas, Hodgkins disease, immunodeficiency, aplastic anemia and lymphohistiocytic disorders that cha

98 omere attrition in bone marrow cells rescues aplastic anemia and mouse survival compared with mice tr

99 lso are significantly lower in patients with aplastic anemia and NFAT1 protein levels are decreased o

100 f novel therapeutic agents for patients with aplastic anemia and other autoimmune diseases.

101 rescue, has been used successfully to treat aplastic anemia and other autoimmune disorders.

102 tion leads to durable complete remissions in aplastic anemia and other autoimmune disorders.

103 kinase may prove useful in the treatment of aplastic anemia and other cytokine-mediated bone marrow

104 enita have shed light on the pathobiology of aplastic anemia and other forms of bone marrow dysfuncti

105 the skin in an 11-year-old child with severe aplastic anemia and prolonged neutropenia is reported.

106 he second featured a 31-year-old female with aplastic anemia and prolonged neutropenia who developed

107 d with telomere dysfunction, including AIDS, aplastic anemia and pulmonary fibrosis, has bolstered in

108 isorder associated with premature death from aplastic anemia and pulmonary fibrosis.

109 ights into the pathophysiology of idiopathic aplastic anemia and suggest new treatment options, becau

110 ave been described in patients with acquired aplastic anemia and the autosomal dominant form of dyske

111 athogenesis of hematologic disorders such as aplastic anemia and the development of neoplasia are bel

112 r patients with hematologic malignancies and aplastic anemia are almost certain to follow up patients

113 ated donor bone marrow transplant for severe aplastic anemia as a manifestation of Schwachman-Diamond

114 emoglobinuria (PNH) is intimately related to aplastic anemia because many patients with bone marrow f

115 ssays in 18 consecutive patients with severe aplastic anemia before and after treatment with high-dos

116 nts of HLA-identical sibling transplants for aplastic anemia between 1976 and 1992, reported to the I

117 , progressive loss of bone marrow, and fatal aplastic anemia between 3 and 4 months of age.

118 hemopoietic progenitor colony formation from aplastic anemia bone marrows in vitro.

119 telomere length have been reported in severe aplastic anemia but their clinical significance is unkno

120 Aplastic anemia can be a presenting manifestation of T-L

121 Aplastic anemia can be effectively treated by stem cell

122 Aplastic anemia can be effectively treated by stem-cell

123 ce polymorphisms found in some patients with aplastic anemia can inhibit telomerase activity by disru

124 st universally fatal just a few decades ago, aplastic anemia can now be cured or ameliorated by stem-

125 sted telomere length of patients with severe aplastic anemia consecutively enrolled in immunosuppress

126 The hepatitis of the hepatitis-associated aplastic anemia does not appear to be caused by any of t

127 e Tert gene in 2 independent mouse models of aplastic anemia due to short telomeres (Trf1- and Tert-d

128 ns associated with dyskeratosis congenita or aplastic anemia either impair the specific activity of t

129 ion, marrow transplantation in patients with aplastic anemia established long-term normal hematopoies

130 mia is a variant of aplastic anemia in which aplastic anemia follows an acute attack of hepatitis.

131 40 patients received transplants for severe aplastic anemia from related donors other than HLA genot

132 cal sibling bone marrow transplantations for aplastic anemia has improved since 1976.

133 anemias are good in vivo models for studying aplastic anemia in general; some of the idiopathic aplas

134 rtant role in the pathogenesis of idiopathic aplastic anemia in humans.

135 His brother had aplastic anemia in the course of his EBV infection and d

136 C partial loss-of-function allele results in aplastic anemia in the homozygous state and mild thrombo

137 tinct cell surface receptor and cause severe aplastic anemia in vivo and erythroblast destruction in

138 s-associated aplastic anemia is a variant of aplastic anemia in which aplastic anemia follows an acut

139 Aplastic anemia is a fatal bone marrow disorder characte

140 Severe aplastic anemia is a life-threatening bone marrow failur

141 Hepatitis-associated aplastic anemia is a variant of aplastic anemia in which

142 Aplastic anemia is caused by several diverse factors, in

143 Aplastic anemia is known to respond to immunosuppressive

144 The pathophysiology of aplastic anemia is now believed to be immune-mediated, w

145 d tumor may become even higher as death from aplastic anemia is reduced and as patients survive longe

146 Most acquired aplastic anemia is the result of immune-mediated destruc

147 Our understanding of the pathophysiology of aplastic anemia is undergoing significant revision, with

148 netically identical twins into patients with aplastic anemia may help define how frequently these fac

149 uced in patients' peripheral blood and in an aplastic anemia murine model, infusion of regulatory T c

150 e more common in developing countries, where aplastic anemia occurs more frequently than it does in t

151 rformed on a limited number of patients with aplastic anemia or acute leukemia, but only transient en

152 tent stem cells (iPSCs) from 4 patients with aplastic anemia or hypocellular bone marrow carrying het

153 rvival after bone marrow transplantation for aplastic anemia or leukemia was poor in both cohorts.

154 cted by high-sensitivity flow cytometry have aplastic anemia or low-risk myelodysplastic syndrome.

155 a may be warranted in selected patients with aplastic anemia or myelodysplastic syndrome, as this may

156 bset of patients presumed to have idiopathic aplastic anemia or myelodysplastic syndrome.

157 rating an immune-mediated process underlying aplastic anemia pathogenesis.

158 t the increased IFN-gamma levels observed in aplastic anemia patients are the result of active transc

159 Bone marrow (BM) and lymphocyte samples from aplastic anemia patients show up-regulated Fas and Fas-l

160 esents a novel therapeutic strategy to treat aplastic anemia provoked or associated with short telome

161 In a cohort of patients with severe aplastic anemia receiving immunosuppressive therapy, tel

162 Most patients with aplastic anemia recover bone marrow function after recei

163 is efficacious in a subset of patients with aplastic anemia refractory to immunosuppressive therapy,

164 patibility in 16 alloimmunized patients with aplastic anemia refractory to random donor platelet tran

165 About a quarter of patients with severe aplastic anemia remain pancytopenic despite immunosuppre

166 siology in the majority of cases of acquired aplastic anemia remains unknown ("idiopathic").

167 Acquired aplastic anemia results from immune-mediated destruction

168 In most patients, aplastic anemia results from T-cell-mediated immune dest

169 d in patients with dyskeratosis congenita or aplastic anemia show loss of function without any indica

170 were noted in analyses stratified on severe aplastic anemia subtype, recipient age, HLA matching, ca

171 ron-gamma promoter region, is upregulated in aplastic anemia T cells.

172 atologic response among patients with severe aplastic anemia than in a historical cohort.

173 al infection, myelodysplasia, lymphedema, or aplastic anemia that progress to myeloid leukemia.

174 In patients with aplastic anemia that was refractory to immunosuppression

175 cted a phase 2 study involving patients with aplastic anemia that was refractory to immunosuppression

176 mance score, graft type, HLA matching, prior aplastic anemia therapy, race/ethnicity, and calendar ye

177 he records and reevaluated 212 patients with aplastic anemia transplanted at the Fred Hutchinson Canc

178 analyzed results in 700 patients with severe aplastic anemia treated with allogeneic marrow transplan

179 Approximately half of patients with severe aplastic anemia treated with antithymocyte globulin and

180 ween 1970 and 1996, 333 patients with severe aplastic anemia underwent HLA-matched related marrow tra

181 Complete remission of aplastic anemia was achieved in four of these five patie

182 Bone marrow transplants for severe aplastic anemia were first performed in the 1970s.

183 even patients with hematologic malignancy or aplastic anemia were prepared to receive a transplant wi

184 Ten patients with hepatitis-associated aplastic anemia were referred to the NIH between 1990 an

185 viduals is highlighted by an individual with aplastic anemia who appears to lack six contiguous IGHD

186 ested that survivors of childhood cancer and aplastic anemia who are infected with the hepatitis C vi

187 serves further study in patients with severe aplastic anemia who are not suitable candidates for allo

188 In 87 patients with aplastic anemia who failed to respond to immunosuppressi

189 me of unrelated transplants in patients with aplastic anemia who had received multiple transfusions.

190 preferred for younger patients with acquired aplastic anemia who have matched, related donors.

191 itution analysis of 183 patients with severe aplastic anemia who were treated in sequential prospecti

192 s of patients with dyskeratosis congenita or aplastic anemia with mutations in telomerase genes can i

193 The patient had aplastic anemia with prolonged neutropenia and was treat

194 mphoma, 1 Chronic Myeloid Leukemia, 2 Severe Aplastic Anemia) undergoing allo-HSCT.

195 than leukemia (odds ratio=6.5 compared with aplastic anemia), and grade 4 graft-versus-host disease

196 a rate similar to that seen in patients with aplastic anemia).

197 115 patients with aplastic anemia, 39 patients with myelodysplasia, 28 pat

198 failure syndromes dyskeratosis congenita and aplastic anemia, acute myeloid leukemia, liver cirrhosis

199 hemoglobinuria is frequently associated with aplastic anemia, although the basis of this relation is

200 Aplastic anemia, an unusual hematologic disease, is the

201 5%) of 26 patients with hepatitis-associated aplastic anemia, and 0 of 17 patients with cryptogenic a

202 progressive immunoglobulin deficiency, FIM, aplastic anemia, and B-cell lymphoma.

203 plain the association between B19 infection, aplastic anemia, and chronic neutropenia of childhood.

204 MECOM patients presented early-onset severe aplastic anemia, and ERCC6L2 patients, mild pancytopenia

205 ggesting mutations in patients with acquired aplastic anemia, and for selection of suitable hematopoi

206 ions associated with dyskeratosis congenita, aplastic anemia, and idiopathic pulmonary fibrosis disru

207 ow failure syndromes dyskeratosis congenita, aplastic anemia, and idiopathic pulmonary fibrosis.

208 ed for children and young adults with severe aplastic anemia, and immunosuppressive therapy is employ

209 dyskeratosis congenita, pulmonary fibrosis, aplastic anemia, and liver fibrosis.

210 sis, thalassemia major, sickle cell disease, aplastic anemia, and myelodysplasia, among others.

211 ompared with other hematologic malignancies, aplastic anemia, and myelodysplastic syndrome.

212 BAF53a resulted in multilineage BM failure, aplastic anemia, and rapid lethality.

213 In patients with post-hepatitis aplastic anemia, antibodies to the known hepatitis virus

214 to horse ATG as a first treatment for severe aplastic anemia, as indicated by hematologic response an

215 ailure, dyskeratosis congenita, and acquired aplastic anemia, both diseases that predispose to acute

216 n associated with dyskeratosis congenita and aplastic anemia, both typified by impaired haemopoietic

217 ty syndrome characterized by childhood-onset aplastic anemia, cancer or leukemia susceptibility, and

218 utosomal recessive disorder characterized by aplastic anemia, cancer susceptibility, and cellular sen

219 utosomal recessive disorder characterized by aplastic anemia, cancer susceptibility, and cellular sen

220 tability disorder characterized by childhood aplastic anemia, developmental abnormalities and cancer

221 ant dyskeratosis congenita (DKC), as well as aplastic anemia, has been linked to mutations in the RNA

222 eratosis congenita, another familial form of aplastic anemia, have a high incidence of hematopoietic

223 Androgens, used in the treatment of aplastic anemia, have been reported to block proliferati

224 those obtained in a series of patients with aplastic anemia, healthy donors, and patients with a his

225 In aplastic anemia, hematopoiesis fails: Blood cell counts

226 In severe acquired aplastic anemia, hematopoietic failure is the result of

227 in chronic inflammatory conditions, such as aplastic anemia, HIV, and graft-versus-host disease, is

228 The aplastic anemia, however, is often fatal if untreated.

229 In aplastic anemia, immune destruction of hematopoietic cel

230 der individual or after recovery from immune aplastic anemia, is uncertain.

231 ening is also described in cases of acquired aplastic anemia, most likely secondary to increased turn

232 Patients with aplastic anemia, myelodysplasia, or renal allografts rec

233 rast, bone marrow from karyotypically normal aplastic anemia, myelodysplastic syndrome, or healthy in

234 dels of marrow failure, and to patients with aplastic anemia, myeloid, and lymphoid cell malignancies

235 In aplastic anemia, oligoclonally expanded cytotoxic T cell

236 utations and the effects of THPO agonists in aplastic anemia, our results have clinical implications

237 h diseases including dyskeratosis congenita, aplastic anemia, pulmonary fibrosis and cancer.

238 mbilical cord blood transplantation and with aplastic anemia, results from insufficient numbers of ea

239 play a critical role in the pathogenesis of aplastic anemia, suggesting that selective pharmacologic

240 Aplastic anemia, the paradigm of immune-mediated bone ma

241 Severe aplastic anemia, which is characterized by immune-mediat

242 uch as sickle cell disease, thalassemia, and aplastic anemia--necessitate chronic transfusion before

243 coexpression of wild-type TERT and TERT with aplastic anemia-associated mutations in a telomerase-def

244 amide is highly effective therapy for severe aplastic anemia.

245 and choice of graft source for patients with aplastic anemia.

246 expression and diminished Treg frequency in aplastic anemia.

247 ng the immunodominant Ag H60, produced fatal aplastic anemia.

248 ns in the perforin gene occurred in acquired aplastic anemia.

249 ) is increased in T cells from patients with aplastic anemia.

250 (total of 99 immunosuppressive courses) with aplastic anemia.

251 osomy 7 in severe congenital neutropenia and aplastic anemia.

252 other components of telomerase also occur in aplastic anemia.

253 e is an additional feature shared by PNH and aplastic anemia.

254 l-depleted inoculum and transplantations for aplastic anemia.

255 nfused with aplasia and can also evolve from aplastic anemia.

256 en with MDS secondary to therapy or acquired aplastic anemia.

257 atients with therapy-related MDS or acquired aplastic anemia.

258 s durable treatment-free remission in severe aplastic anemia.

259 myelodysplasia and one of four patients with aplastic anemia.

260 h hematopoietic malignancy or progression to aplastic anemia.

261 e associated with dyskeratosis congenita and aplastic anemia.

262 atients, T-LDGL is reported as presenting as aplastic anemia.

263 cal characteristics were similar to acquired aplastic anemia.

264 ed in the differential diagnosis of acquired aplastic anemia.

265 n cases of leukopenia, thrombocytopenia, and aplastic anemia.

266 H was found in 25 of 115 (22%) patients with aplastic anemia.

267 , providing a unique insight into a cause of aplastic anemia.

268 thality due to acute bone marrow failure and aplastic anemia.

269 degree of neutropenia or a prior history of aplastic anemia.

270 le, complete remission in most patients with aplastic anemia.

271 diseases, such as dyskeratosis congenita and aplastic anemia.

272 ay be relevant to the pathogenesis of MDS in aplastic anemia.

273 on are accepted treatments for patients with aplastic anemia.

274 red immunodeficiency syndrome, and 1 case of aplastic anemia.

275 al disorders, as do patients with idiopathic aplastic anemia.

276 sis is also seen in patients with idiopathic aplastic anemia.

277 in previously untreated patients with severe aplastic anemia.

278 ival in patients who received HCT for severe aplastic anemia.

279 ytic leukemic, myelodysplastic syndrome, and aplastic anemia.

280 flicted with idiopathic, autosomal recessive aplastic anemia.

281 nomucor elegans in a patient with refractory aplastic anemia.

282 nses in some patients with refractory severe aplastic anemia.

283 an, phase 2 pilot study, in 35 patients with aplastic anemia.

284 telomere length (LTL) and increased risk for aplastic anemia.

285 transfusion, such as sickle cell disease and aplastic anemia.

286 une thrombocytopenia, Evans syndrome, severe aplastic anemia/refractory cytopenia, and others.

287 at presentation in almost all patients with aplastic anemia; FOXP3 protein and mRNA levels also are

288 great majority of young patients with severe aplastic anemia; the major challenges are extending the

289 Therefore, the familial aplastic anemias are good in vivo models for studying ap

290 ic anemia in general; some of the idiopathic aplastic anemias could prove to be due to mutations in g

291 Acquired and congenital aplastic anemias recently have been linked molecularly a

292 n genes characterized originally in familial aplastic anemias.

293 coni's anemia, the commonest of the familial aplastic anemias.

294 ypes of Tbx1 heterozygotes as hypoplastic or aplastic at the conclusion of pharyngeal artery formatio

295 eomalacia and, more recently, by adynamic or aplastic bone disease.

296 infection is the primary cause of transient aplastic crisis.

297 ds in mice aged 80 d bore claudin-4-positive aplastic lesions and accumulated (111)In-cCPE.GST (3.17

298 al syndrome characterized by microphthalmia, aplastic skin and agenesis of the corpus callosum, and i

299 required to switch the mature neuron from an aplastic state to a state capable of growth.

300 arian strip may play a role in governing the aplastic transport of these elements.