ライフサイエンスコーパス: AL amyloidosis

1 A) or monoclonal immunoglobulin light chain (AL amyloidosis).

2 will help develop risk-adapted therapies for AL amyloidosis.

3 S were significant predictors of survival in AL amyloidosis.

4 mide, cyclophosphamide, and dexamethasone in AL amyloidosis.

5 ntribute to the site selectivity observed in AL amyloidosis.

6 volvement were assessed in 191 patients with AL amyloidosis.

7 the diagnosis and treatment of patients with AL amyloidosis.

8 for patients with other dominant features of AL amyloidosis.

9 contribution of autoimmunity in this type of AL amyloidosis.

10 at lenalidomide can be effective in treating AL amyloidosis.

11 ion with dexamethasone, for the treatment of AL amyloidosis.

12 thogenesis of the neuropathy associated with AL amyloidosis.

13 c, mild cardiac, and severe cardiac involved AL amyloidosis.

14 in a substantial proportion of patients with AL amyloidosis.

15 of a patient diagnosed with kappa 1 (kappa1) AL amyloidosis.

16 otential involvement of this modification in AL amyloidosis.

17 from the spleens or livers of patients with AL amyloidosis.

18 ciation of monoclonal lambda 6 proteins with AL amyloidosis.

19 and therefore, we evaluated its efficacy for AL amyloidosis.

20 he genesis of heart failure in patients with AL amyloidosis.

21 LC dimer could be a useful strategy to treat AL amyloidosis.

22 are limited data on endothelial function in AL amyloidosis.

23 ntigens in 111 newly diagnosed patients with AL amyloidosis.

24 .60 after MGUS, 1.76 after WM and 2.18 after AL amyloidosis.

25 usively from uptake in patients with cardiac AL amyloidosis.

26 e is reshaping the approach to patients with AL amyloidosis.

27 ed with improved biomarker response rates in AL amyloidosis.

28 potential new therapy for the management of AL amyloidosis.

29 ruitment, and cytotoxicity that may occur in AL amyloidosis.

30 1.70 after MM, 1.85 after WM and 2.31 after AL amyloidosis.

31 important independent prognostic factors in AL amyloidosis.

32 has become a cornerstone in the treatment of AL amyloidosis.

33 light chains before clinical presentation of AL amyloidosis.

34 esent a novel clinicopathological pattern of AL amyloidosis.

35 responding, heavily pretreated patients with AL amyloidosis.

36 onses and promising long-term OS in relapsed AL amyloidosis.

37 humanized 2B6 MoAb in patients with systemic AL-amyloidosis.

38 tory drugs are active agents in light-chain (AL) amyloidosis.

39 ise in the treatment of amyloid light-chain (AL) amyloidosis.

40 ohort of patients with systemic light-chain (AL) amyloidosis.

41 c parameter in systemic amyloid light chain (AL) amyloidosis.

42 of patients with immunoglobulin light chain (AL) amyloidosis.

43 determines prognosis in amyloid light-chain (AL) amyloidosis.

44 apsed/refractory immunoglobulin light chain (AL) amyloidosis.

45 e needed to clarify its role in light chain (AL) amyloidosis.

46 ry systemic amyloid light chain amyloidosis (AL) amyloidosis.

47 LS was more depressed in both ATTRwt and AL amyloidosis (-11+/-3% and -12+/-4%, respectively, P=0

48 a significant effect on overall survival in AL amyloidosis (16.2 months vs. 1.4 months; p = 0.003).

49 Among 701 patients with AL amyloidosis, 394 (56%) were eligible for high-dose me

50 age range, 24-88 years) and 67 patients with AL amyloidosis (43 men, 24 women; median age, 65 years;

51 Sixty patients with systemic AL amyloidosis (65% men, median age 65 years) underwent

52 rapy for patients with systemic light-chain (AL-) amyloidosis, a protein deposition and monoclonal pl

53 At least some patients with AL amyloidosis achieve complete remission of their plasm

54 Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators.

55 d to adult patients with relapsed/refractory AL amyloidosis after 1 or more prior lines of therapy (i

56 rs or more after the histologic diagnosis of AL amyloidosis; all received alkylating-agent therapy.

57 In immunoglobulin light chain (AL) amyloidosis, amyloid fibril deposits derived from im

58 0 with non-ATTR cardiac amyloidosis [34 with AL amyloidosis and 16 with nonamyloid heart failure with

59 Both AL amyloidosis and 2 of 10 TTR-noPN subjects were Congo

60 0.54) than in ATTRm (-15+/-4%, P<0.01 versus AL amyloidosis and ATTRwt).

61 Patients with AL amyloidosis and congestive heart failure have a very

62 Seven patients with AL amyloidosis and controls were studied.

63 y ASCT is feasible in selected patients with AL amyloidosis and heart failure, and that such a strate

64 ve had a favorable effect in 3 patients with AL amyloidosis and heart failure.

65 iac involvement predicts outcome in systemic AL amyloidosis and influences therapeutic options.

66 6 transgenic model replicates the process of AL amyloidosis and is useful for testing the antifibril

67 is an effective combination for treatment of AL amyloidosis and leads to durable hematologic response

68 s feasible in selected patients with cardiac AL amyloidosis and may confer substantial survival benef

69 d as an independent predictor of survival in AL amyloidosis and offered incremental information beyon

70 ne its diagnostic potential in patients with AL amyloidosis and other systemic amyloidoses.

71 /expansion study of NEOD001 in patients with AL amyloidosis and persistent organ dysfunction (NCT0170

72 owed by SCT was performed in 5 patients with AL amyloidosis and predominant cardiomyopathy.

73 ed plasma cells from a patient (Wil) who had AL amyloidosis and renal amyloid deposits; the second wa

74 proves risk stratification for patients with AL amyloidosis and will help develop risk-adapted therap

75 e, to our knowledge, the association between AL-amyloidosis and AION was not previously described.

76 markedly better than observed with systemic AL amyloidosis, and outcomes with renal replacement ther

77 ously reported nontransplantation regimen in AL amyloidosis, and risk adaptation allows its use in po

78 t observed in patients with cardiac involved AL amyloidosis, and they suggest that amyloid LC protein

79 ment predicts poor prognosis in light chain (AL) amyloidosis, and the current prognostic classificati

80 Light chain (LC) amyloidosis (AL amyloidosis) appears to be caused by the misfolding,

81 eria of hematologic response in light-chain (AL) amyloidosis are based on the measurement of circulat

82 strom macroglobulinemia (WM) and light chain AL amyloidosis, are characterized by clonal expansion of

83 stemic amyloidogenic light chain-associated (AL) amyloidosis, are presumed to be the source of light

84 AL amyloidosis associated with immunoglobulin M (IgM) pa

85 oves the nephrotic syndrome in patients with AL amyloidosis-associated renal disease.

86 Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hyp

87 MFC is prognostic for AL amyloidosis at diagnosis and at EOT.

88 Compared with ATTRm and AL amyloidosis, ATTRwt was characterized by greater LV w

89 eria for response to first-line treatment in AL amyloidosis, based on their association with survival

90 dosis can be misdiagnosed as Ig light-chain (AL) amyloidosis because family history is an ineffective

91 undetermined significance who are developing AL amyloidosis before they become symptomatic.

92 all Mayo Clinic patients with a diagnosis of AL amyloidosis between January 1, 1966 and March 1, 1987

93 cardiographic studies have been performed in AL amyloidosis but not in TTR amyloidosis and might give

94 ation (ASCT) has become a common therapy for AL amyloidosis, but there is an exceedingly high treatme

95 of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or r

96 ffective treatment for systemic light-chain (AL) amyloidosis, but many patients are ineligible becaus

97 Patients were diagnosed with AL amyloidosis by tissue biopsy and categorized by perfo

98 Treatment of selected patients with AL amyloidosis by using high-dose melphalan and stem-cel

99 ont-line therapy with a DEX-based regimen in AL amyloidosis can lead to durable reversal of AL amyloi

100 t of the underlying plasma cell dyscrasia in AL amyloidosis can lead to the amelioration of amyloid-r

101 oidosis, which accounts for 6% to 10% of all AL amyloidosis cases, is a rare and poorly studied clini

102 or 65 patients (aged 65 years or older) with AL amyloidosis compared with outcomes for 280 younger pa

103 Care of patients with AL amyloidosis currently is limited by the lack of objec

104 ickness but lesser mortality than those with AL amyloidosis, despite very similar degrees of LS impai

105 Of 368 consecutive patients with systemic AL amyloidosis evaluated at Boston Medical Center, 32 pa

106 upport is feasible therapy for patients with AL amyloidosis, even when there is clinical evidence of

107 ed normal range, precedes the development of AL amyloidosis for many years.

108 ment outcomes of 346 patients with stage III AL amyloidosis from the United Kingdom, Italy, Germany,

109 with immunoglobulin light chain amyloidosis (AL amyloidosis) generally present with advanced organ dy

110 ntification of this atypical presentation of AL amyloidosis has important implications for early dete

111 Survival in AL amyloidosis has improved markedly as novel chemothera

112 development of less intensive treatments for AL amyloidosis has made less certain the role of autolog

113 The management of light chain (AL) amyloidosis has improved in recent years thanks to a

114 flow cytometry (MFC) in amyloid light-chain (AL) amyloidosis has not been widely adopted and, consequ

115 Primary systemic (amyloid light-chain [AL]) amyloidosis has a variety of cutaneous manifestatio

116 Patients with IgM AL amyloidosis have a significant IgM paraprotein (media

117 Previous reports of PXE-like plaques in AL amyloidosis have been reported as part of a very rare

118 Overall, outcomes among patients with AL amyloidosis have improved with earlier diagnosis, hig

119 een percent of newly diagnosed patients with AL amyloidosis have low dFLC and had a better outcome.

120 No published series of patients with AL amyloidosis have reported survival of more than 10 ye

121 a monoclonal component in patients with non-AL amyloidosis, highlighting the risk of misdiagnosis an

122 used in multiple myeloma can be effective in AL amyloidosis; however, patients with this disease ofte

123 led behind the progress made in the field of AL amyloidosis in diagnosis, prognosis, and hematologic

124 Prognostic and response criteria for AL amyloidosis in general have not been validated in thi

125 Use of BDex+AA in the treatment of AL amyloidosis in patients presenting with symptomatic h

126 are discussed, with particular reference to AL amyloidosis in the heart.

127 o have high sensitivity in imaging of AA and AL amyloidosis in visceral organs.

128 ivariate analysis, predictors of survival in AL amyloidosis included sex, Karnofsky index, New York H

129 ated CTD (CTDa) in 75 patients with advanced AL amyloidosis, including 44 patients with clonal relaps

130 e associated with prognosis in patients with AL amyloidosis, independently of other features of the d

131 IgM AL amyloidosis is a distinct clinical entity.

132 AL amyloidosis is a fatal disease resulting from tissue

133 nsive cardiac amyloid deposition in systemic AL amyloidosis is associated with a grave prognosis.

134 AL amyloidosis is associated with a high incidence of ca

135 The morbidity and lethality of AL amyloidosis is caused by the deposition of lg light c

136 Survival of patients with AL amyloidosis is no more than 1 to 2 years from the tim

137 Although cardiac death in patients with AL amyloidosis is usually associated with extensive myoc

138 Primary (AL) amyloidosis is a plasma cell dyscrasia characterized

139 Amyloid light chain (AL) amyloidosis is a protein misfolding disease where im

140 Immunoglobulin light-chain (AL) amyloidosis is a rare, incurable plasma cell disorde

141 Light chain (AL) amyloidosis is an incurable human disease characteri

142 Light chain (AL) amyloidosis is caused by a usually small plasma cell

143 Light chain (AL) amyloidosis is caused by the accumulation of misfold

144 Systemic light chain (AL) amyloidosis is caused by the clonal production of an

145 Primary light-chain-associated (AL) amyloidosis is characterized by the deposition in ti

146 Light chain (AL) amyloidosis is characterized by the misfolding of im

147 Light chain (or AL) amyloidosis is characterized by the pathological dep

148 Amyloid-deposited light chain (AL) amyloidosis is correlated with the overproduction of

149 The median survival in primary systemic (AL) amyloidosis is less than 18 months.

150 Light chain (or AL) amyloidosis is the most common form of systemic amyl

151 Systemic light chain (AL) amyloidosis is the most common of these conditions,

152 osis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease.

153 Light chain, or AL, amyloidosis is a pathological condition arising from

154 In amyloid light-chain (AL) amyloidosis, kappa cases were more difficult to diag

155 A woman in her 50s with a history of AL amyloidosis manifesting as macroglossia and bilateral

156 It is known that light-chain (AL) amyloidosis may rarely affect the temporal arteries,

157 The present case shows that AL-amyloidosis may present with AION, high ESR, and temp

158 Since the amyloid fibril deposits in AL amyloidosis most often consist of the N-terminal frag

159 Thirty-four patients with AL amyloidosis, most with prior therapies, were enrolled

160 We analyzed 172 patients with CA (AL amyloidosis, n=80; ATTRm, n=36; ATTRwt, n=56) by stan

161 y (AION) showing histopathologic evidence of AL-amyloidosis of the temporal arteries.

162 mains in the form of either amyloid fibrils (AL-amyloidosis) or amorphous deposits, light-chain depos

163 lation in the forearm skin was attenuated in AL amyloidosis patients (p = 0.007).

164 s and their associated variable domains from AL amyloidosis patients and non-patients.

165 Early in the disease, AL amyloidosis patients present with impaired endothelia

166 ment, and outcome among 1551 newly diagnosed AL amyloidosis patients seen in our institution from 200

167 Intrigued by the unique response rates of AL amyloidosis patients to the first-in-class proteasome

168 Full-length LC dimers derived from AL amyloidosis patients unfold more rapidly than other f

169 Thus, treatment of AL amyloidosis patients with HDM/SCT produces measurable

170 Treatment of AL amyloidosis patients with high-dose melphalan chemoth

171 f the full-length LC dimers, even those from AL amyloidosis patients, are amyloidogenic.

172 unction in the cutaneous microcirculation of AL amyloidosis patients.

173 patients with systemic amyloid light-chain (AL) amyloidosis, presumably due to adsorption of factor

174 aracterized the internalization of AL-09, an AL amyloidosis protein into mouse cardiomyocytes.

175 and its modulation in patients with systemic AL-amyloidosis receiving high-dose melphalan.

176 amyloidosis can lead to durable reversal of AL amyloidosis-related organ dysfunction and prolonged s

177 ions were observed in 24% and improvement in AL amyloidosis-related organ dysfunction occurred in 45%

178 ch domain and the role of endoproteolysis in AL amyloidosis remain unclear.

179 patients with cardiac stage III light chain (AL) amyloidosis remain poorly studied.

180 Light-chain (AL) amyloidosis remains incurable despite recent therape

181 show a survival advantage for patients with AL amyloidosis responding to salvage treatment with poma

182 trate that infusion of LC from patients with AL amyloidosis result in diastolic dysfunction similar t

183 Systemic AL amyloidosis results from the aggregation of an amyloi

184 A 64 year-old woman with AL-amyloidosis secondary to a monoclonal gammopathy of u

185 nostic value independent of age, Mayo Clinic AL amyloidosis stage, prior autologous stem-cell transpl

186 n additional mechanism for LV dysfunction in AL amyloidosis, such as previously demonstrated light-ch

187 Five percent of patients with AL amyloidosis survived for 10 years or more.

188 To characterize symptoms and signs of AL amyloidosis that may bring patients to the attention

189 essment of renal response and progression in AL amyloidosis that should be used in clinical practice

190 ctive regimen producing durable responses in AL amyloidosis; the deep clonal responses may overcome p

191 ng patients with immunoglobulin light chain (AL) amyloidosis, there is little consensus on when reins

192 tructures, and bones can bring patients with AL amyloidosis to the attention of rheumatologists.

193 tudies have suggested that, in patients with AL amyloidosis treated with high-dose melphalan and auto

194 80 patients with immunoglobulin light chain (AL) amyloidosis treated with high-dose melphalan and ste

195 tic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic re

196 ystem for patients with amyloid light-chain (AL) amyloidosis using a test set of 461 patients from Pa

197 Patient survival in AL amyloidosis was 8.9 years among those who had achieve

198 ent staging and response criteria in non-IgM AL amyloidosis was applied to this series to assess its

199 pa1 LC purified from urine of a patient with AL amyloidosis was incubated in the presence or absence

200 ajor advances in immunoglobulin light chain (AL) amyloidosis, we evaluated the trends in presentation

201 Ninety-seven biopsy-proven patients with AL amyloidosis were divided into 3 groups.

202 996 and 2003, 93 patients with biopsy-proven AL amyloidosis were enrolled in a prospective US nationa

203 six consecutive patients with biopsy-proven AL amyloidosis were investigated in this prospective obs

204 functional class >/=II heart failure due to AL amyloidosis were retrospectively studied.

205 the 21 years of the study, 841 patients with AL amyloidosis were seen.

206 amethasone for the treatment of light chain (AL) amyloidosis were to determine the safety, tolerabili

207 -chain variable domain, SMA, associated with AL amyloidosis, were investigated by (15)N relaxation di

208 Immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 6% to 10% of all AL

209 This amyloid light-chain (AL) amyloidosis, which is a hereditary type of amyloidos

210 esents a distinctive subset of patients with AL amyloidosis who have a wider variety of underlying cl

211 Patients with AL amyloidosis who have more than 10% BMPCs have a poor

212 d as the preferred therapy for patients with AL amyloidosis who meet functional criteria for autologo

213 Patients with AL amyloidosis who need second-line therapy after respon

214 e present our experience of 43 patients with AL amyloidosis who received cyclophosphamide, bortezomib

215 We studied 173 patients with AL amyloidosis who underwent MFC immunophenotyping of bo

216 de and dexamethasone (PDex) in patients with AL amyloidosis who were previously exposed to bortezomib

217 atients with congestive heart failure due to Al amyloidosis who were seen between 1983 and 1994.

218 Patients with light chain (AL) amyloidosis who present with severe heart failure du

219 tment plasma cells of patients with systemic AL-amyloidosis who then had a complete response to high-

220 as to examine the spectrum of immunoglobulin AL amyloidosis with and without MM, with a goal of defin

221 AL amyloidosis with heart failure is associated with dec

222 AL amyloidosis with IgM paraproteinemia represents a dis

223 rrow plasma cell (BMPC) number to qualify as AL amyloidosis with MM.

224 d should therefore be considered together as AL amyloidosis with MM.

225 tested the associations of MGUS, MM, WM and AL amyloidosis with subsequent eye diseases identified f

226 is consensus that patients with light chain (AL) amyloidosis with hypercalcemia, renal failure, anemi

227 ons characterize immunoglobulin light-chain (AL) amyloidosis with major heart involvement.

228 Current therapy of primary systemic (AL) amyloidosis with oral melphalan and prednisone remai

229 active in patients with relapsed/refractory AL amyloidosis, with a generally manageable safety profi

230 redict for 1-year mortality in patients with AL amyloidosis without CRAB to produce two additional gr

231 Outcome and organ function of stage III AL amyloidosis without very elevated NT-proBNP and low S