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1.
Int Heart J ; 60(5): 1106-1112, 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31484874

RESUMO

A useful biomarker for detecting cardiac amyloidosis (CA) has not been fully established. We aimed to investigate the utility of several biomarkers to detect CA in patients with amyloid light-chain (AL) amyloidosis.We examined the plasma levels of B-type natriuretic peptide (BNP), N-terminal fragment of the pro-brain natriuretic peptide (NT-proBNP), high-sensitivity cardiac troponin T (hs-cTnT), serum amyloid A, and the difference between kappa and lambda free light chain (dFLC) between CA patients (n = 30, 47.6%) and non-CA patients (n = 33, 52.4%). Levels of BNP were significantly higher in the CA group compared to the non-CA group (1200.0 versus 224.0 pg/mL, P = 0.001). From the ROC analysis, the sensitivity and specificity of BNP for detecting CA (with a cut-off value of 412 pg/mL) were 83% and 70%, respectively, and the area under the receiver operating curve was 0.75 (95% CI 0.61-0.90, P < 0.001) in all AL amyloidosis patients (n = 63). In contrast, other markers such as NT-proBNP, hs-cTnT, serum amyloid A, and dFLC were not useful for detecting CA in AL amyloidosis patients. Additionally, in the Cox proportional hazard analysis, BNP was a predictor of all-cause mortality (hazard ratio 3.266, 95% confidence interval 1.498-7.119, P = 0.003).BNP is a useful biomarker for detecting cardiac involvement and predicting prognosis in AL amyloidosis patients.


Assuntos
Cardiopatias/sangue , Cardiopatias/epidemiologia , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Peptídeo Natriurético Encefálico/sangue , Troponina T/sangue , Idoso , Biomarcadores/sangue , Causas de Morte , Estudos de Coortes , Progressão da Doença , Ecocardiografia Doppler/métodos , Eletrocardiografia/métodos , Feminino , Cardiopatias/diagnóstico por imagem , Hospitais Universitários , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Japão , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida
2.
Ann Biol Clin (Paris) ; 77(4): 397-406, 2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31418701

RESUMO

Immunoglobulin light chains are called free when they are not linked with heavy chains to form a whole immunoglobulin. Quantification of free light chains is a part of the French national authority for health guidelines for diagnostic and follow-up of light chain, oligo or non-secretory myeloma and AL amyloidosis. Most recently, the World health organisation had included free light chains quantification in prognostic criteria for monoclonal gammopathy of undetermined significance. However the literature bring to light some other potential indications of this analysis in the exploration of monoclonal gammopathy, also in lymphoid malignancies and some autoimmune diseases such as diabetes, rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis and Sjögren syndrome.


Assuntos
Doenças Autoimunes/diagnóstico , Neoplasias Hematológicas/diagnóstico , Cadeias Leves de Imunoglobulina/sangue , Paraproteinemias/diagnóstico , Testes Sorológicos , Doenças Autoimunes/sangue , Doenças Autoimunes/imunologia , Diagnóstico Diferencial , Neoplasias Hematológicas/sangue , Neoplasias Hematológicas/imunologia , Humanos , Cadeias Leves de Imunoglobulina/análise , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/sangue , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Paraproteinemias/sangue , Paraproteinemias/imunologia , Valor Preditivo dos Testes , Prognóstico , Testes Sorológicos/métodos , Testes Sorológicos/normas
4.
Amyloid ; 26(3): 128-138, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31172799

RESUMO

Background: Atrial fibrillation (AF) commonly affects patients with cardiac amyloidosis (CA). Amyloid deposition within the left atrium may be responsible for the subtype of AF in either permanent or non-permanent form. The prognostic implications of AF and its clinical subtype according to the type of CA are still controversial in this population. This study sought to investigate the prevalence, incidence and prognostic implications of AF and the clinical subtype of AF (permanent or non-permanent) in patients with CA. Methods: Two hundred and thirty-eight patients with CA and full medical records were retrospectively enrolled in the study: About 115 (48%) with light chain (AL) amyloidosis and 123 (52%) with transthyretin amyloidosis (ATTR). Patient's medical records were reviewed to establish baseline prevalence, incidence and impact on all-cause and cardiovascular mortality during follow-up of AF. Results: One hundred and four (44%) patients had history of AF at the time of diagnosis: 62 (60%) permanent and 42 (40%) non-permanent. There were 30 (26%) and 74 (60%) patients with history of AF among patients with AL and ATTR (including 5 hereditary and 69 wild-type), respectively (p<.0001). During the follow-up, 48 new patients developed AF (29, 12 and 7 among patients with AL, wild-type ATTR and hereditary ATTR). After adjustment for age, survival was similar in patients with or without history of AF (HR 0.87 (95% CI, 0.60 to 1.27; p = .467). AF had no impact on cardiovascular mortality. Among the 152 patients with history of AF included in the whole study, there were 75 (49%) patients with permanent AF. After adjustment for age, survival was similar in patients with permanent and non-permanent AF: HR 1.29 (95% CI, 0.84 to 1.99; p = .251). The results were the same among patients with AL or wild-type amyloidosis. Subtype of AF had no impact on cardiovascular mortality. Conclusions: AF is common in patients with CA. However, AF and clinical subtype of AF have no impact on all-cause mortality, whatever the type of amyloidosis.


Assuntos
Neuropatias Amiloides Familiares/diagnóstico por imagem , Fibrilação Atrial/diagnóstico por imagem , Átrios do Coração/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/sangue , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/mortalidade , Fibrilação Atrial/sangue , Fibrilação Atrial/complicações , Fibrilação Atrial/mortalidade , Ecocardiografia , Feminino , Átrios do Coração/fisiopatologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Pré-Albumina/metabolismo , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida
5.
Amyloid ; 26(3): 125-127, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31145007

RESUMO

Objectives: We aimed to externally validate Lilleness' et al. Boston University (BU) prognostic score that replaced NT-proBNP with brain natriuretic peptide (BNP), which will allow centres without access to NT-proBNP to accurately stage and prognosticate AL amyloidosis. Patients/methods: Forty-four were identified that had BNP, NTpro-BNP and TnI taken simultaneously, with a mean follow up of 7.3 years. Median age of the 44 patients was 67 years and 27% were female, with 61% having cardiac involvement, and 61% having renal involvement. Results: Using the BU BNP-based staging system, we identified 12/44 (27%) of patients as stage I, 18/44 (41%) of patients as stage II and 14/44 (31%) of patients as stage III. This correlated closely with stratification via the Mayo score, with only one patient miscategorised (97.7% agreement, k = 0.98). Median overall survival for our BU stage I was not reached, stage II was 40 months and stage III was 5 months (long rank p = .0012). Mayo 2004 median overall survival was identical for stages I, II and III. Conclusion: We have provided external validation of the BU staging system, a novel prognostic scoring system incorporating BNP, instead of NT-proBNP, for AL amyloidosis.


Assuntos
Cardiomiopatias/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Peptídeo Natriurético Encefálico/sangue , Nefrite/diagnóstico , Fragmentos de Peptídeos/sangue , Troponina I/sangue , Idoso , Biomarcadores/sangue , Boston , Cardiomiopatias/sangue , Cardiomiopatias/complicações , Cardiomiopatias/mortalidade , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Nefrite/sangue , Nefrite/complicações , Nefrite/mortalidade , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Análise de Sobrevida , Universidades
6.
Hematol Oncol Stem Cell Ther ; 12(1): 10-14, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30879471

RESUMO

INTRODUCTION: Systemic light chain (AL) amyloidosis can lead to an acquired coagulopathy secondary to acquired factor X (aFX) deficiency. However, it is not very clear who develops aFX deficiency in AL amyloidosis. METHODS: We therefore undertook this single centre, retrospective study to better characterize AL amyloidosis-associated aFX deficiency. RESULTS: Out of 121 AL patients who had FX testing at the time of their first evaluation at our institution, including 17 patients on warfarin at the time of testing, 10 out of 104 patients (9.6%) with systemic AL amyloidosis were found to have FX levels below 50%. Acquired FX deficiency was associated with advanced stage of AL amyloidosis and elevated cardiac biomarkers. Lower FX activity, advanced stage, and cardiac involvement by disease were associated with higher hazard of death on univariate analysis. On multivariate analysis, stage of AL amyloidosis was the only significant predictor of survival. Median survival time of patients with FX deficiency was 9.3 months compared to 118.4 months in those without. CONCLUSIONS: We conclude that while aFX deficiency is rare in systemic AL amyloidosis, it is a marker of advanced disease.


Assuntos
Deficiência do Fator X , Fator X/metabolismo , Amiloidose de Cadeia Leve de Imunoglobulina , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Deficiência do Fator X/sangue , Deficiência do Fator X/etiologia , Deficiência do Fator X/mortalidade , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida
9.
Blood ; 133(3): 215-223, 2019 01 17.
Artigo em Inglês | MEDLINE | ID: mdl-30333122

RESUMO

Immunoglobulin light chain amyloidosis (AL amyloidosis) is caused by misfolded light chains that form soluble toxic aggregates that deposit in tissues and organs, leading to organ dysfunction. The leading determinant of survival is cardiac involvement. Current staging systems use N-terminal pro-brain natriuretic peptide (NT-proBNP) and cardiac troponins T and I (TnT and TnI) for prognostication, but many centers do not offer NT-proBNP. We sought to derive a new staging system using brain natriuretic peptide (BNP) that would correlate with the Mayo 2004 staging system and be predictive for survival in AL amyloidosis. Two cohorts of patients were created: a derivation cohort of 249 consecutive patients who had BNP, NT-proBNP, and TnI drawn simultaneously to create the staging system and a complementary cohort of 592 patients with 10 years of follow-up to determine survival. In the derivation cohort, we found that a BNP threshold of more than 81 pg/mL best associated with Mayo 2004 stage and also best identified cardiac involvement. Three stages were developed based on a BNP higher than 81 pg/mL and a TnI higher than 0.1 ng/mL and compared with Mayo 2004 with high concordance (κ = 0.854). In the complementary cohort, 25% of patients had stage I, 44% had stage II, 15% had stage III, and 16% had stage IIIb disease with a median survival not reached in stage I, 9.4 years in stage II, 4.3 years in stage III, and 1 year in stage IIIb. This new Boston University biomarker scoring system will allow centers without access to NT-proBNP the ability to appropriately stage patients with AL amyloidosis. This trial was registered at www.clinicaltrials.gov as #NCT00898235.


Assuntos
Biomarcadores Tumorais/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Troponina T/sangue , Idoso , Feminino , Seguimentos , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/classificação , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos , Taxa de Sobrevida
12.
BMC Cardiovasc Disord ; 18(1): 221, 2018 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-30509186

RESUMO

BACKGROUND: Cardiac Amyloidosis (CA) pertains to the cardiac involvement of a group of diseases, in which misfolded proteins deposit in tissues and cause progressive organ damage. The vast majority of CA cases are caused by light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). The increased awareness of these diseases has led to an increment of newly diagnosed cases each year. METHODS: We performed multiple searches on MEDLINE, EMBASE and the Cochrane Database of Systematic Reviews. Several search terms were used, such as "cardiac amyloidosis", "diagnostic modalities cardiac amyloidosis" and "staging cardiac amyloidosis". Emphasis was given on original articles describing novel diagnostic and staging approaches to the disease. RESULTS: Imaging techniques are indispensable to diagnosing CA. Novel ultrasonographic techniques boast high sensitivity and specificity for the disease. Nuclear imaging has repeatedly proved its worth in the diagnostic procedure, with efforts now focusing on standardization and quantification of amyloid load. Because the latter would be invaluable for any staging system, those spearheading research in magnetic resonance imaging of the disease are also trying to come up with accurate tools to quantify amyloid burden. Staging tools are currently being developed and validated for ATTR CA, in the spirit of the acclaimed Mayo Staging System for AL. CONCLUSION: Cardiac involvement confers significant morbidity and mortality in all types of amyloidosis. Great effort is made to reduce the time to diagnosis, as treatment in the initial stages of the disease is tied to better prognosis. The results of these efforts are highly sensitive and specific diagnostic modalities that are also reasonably cost effective.


Assuntos
Neuropatias Amiloides Familiares/sangue , Neuropatias Amiloides Familiares/diagnóstico por imagem , Cardiomiopatias/sangue , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Imagem por Ressonância Magnética , Tomografia Computadorizada de Emissão , Biomarcadores/sangue , Humanos , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença
13.
BMC Nephrol ; 19(1): 337, 2018 11 22.
Artigo em Inglês | MEDLINE | ID: mdl-30466387

RESUMO

BACKGROUND: Immunoglobulin heavy-and-light-chain amyloidosis (AHL amyloidosis) is a newly established disease entity where both the immunoglobulin heavy-chain and light-chain compose amyloid fibrils. The immunoglobulins responsible for the amyloid fibrils are generally identified by immunostaining and/or laser microdissection-liquid chromatography-tandem mass spectrometry (LMD-LC-MS/MS). However, both techniques do not biochemically differentiate immunoglobulins that formed amyloid fibrils from non-responsible immunoglobulins. CASE PRESENTATION: We herein report a case of 67-year-old female patient with renal amyloidosis due to lymphoplasmacytic lymphoma secreting monoclonal immunoglobulin M (IgM)-kappa. Renal immunostaining monotypically positive for IgM-kappa and LMD-LC-MS/MS identification of mu heavy-chain and kappa light-chain were consistent with the diagnosis of AHL amyloidosis. In order to confirm that both the immunoglobulin heavy-chain and light-chain were forming amyloid fibrils, we performed LC-MS/MS of renal amyloid fibrils isolated by the traditional amyloid purification method. The additional LC-MS/MS identified kappa light-chain only without any heavy-chain component. These results were suggestive that amyloid fibrils were composed by kappa light-chain only and that the mu heavy-chain identified by immunostaining and LMD-LC-MS/MS was derived from the non-specific co-deposition of monoclonal IgM-kappa. CONCLUSION: The case was AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition. While immunostaining and LMD-LC-MS/MS are irreplaceable techniques to classify amyloidosis, confident exclusion of the present condition should be required to diagnose AHL amyloidosis.


Assuntos
Cadeias Pesadas de Imunoglobulinas/sangue , Cadeias Leves de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos
14.
Ann Biol Clin (Paris) ; 76(5): 575-578, 2018 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-30226197

RESUMO

The presence of serum monoclonal IgM is often associated with the diagnosis of Waldenström macroglobulinemia (WM) or other chronic lymphoproliferative disorders. IgM myeloma is a rare entity (0.5%). We report the case of an IgM myeloma complicated by systemic amyloidosis AL, with an impure nephrotic syndrome and a factor FX deficiency.


Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Imunoglobulina M , Mieloma Múltiplo/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Imunoglobulina M/sangue , Mieloma Múltiplo/sangue , Mieloma Múltiplo/imunologia , Macroglobulinemia de Waldenstrom/diagnóstico
15.
Ann Hematol ; 97(12): 2465-2470, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30056579

RESUMO

To summarize distinct clinical characteristics and prognoses associated with and validate the novel hematologic response criteria in Chinese light-chain amyloidosis patients with a difference between involved and uninvolved free light chain (dFLC) < 50 mg/L. We retrospectively compared clinical features and outcomes between patients in the dFLC < 50 mg/L group (n = 74) and the ≥ 50 mg/L group (n = 248). Patients with dFLC < 50 mg/L presented less frequent and less severe cardiac involvement, but higher renal involvement. Additionally, more patients in the dFLC < 50 mg/L group showed intact immunoglobulin monoclonal protein and high immunoglobulin monoclonal protein levels. Moreover, patients in the dFLC < 50 mg/L group had significantly superior progression-free survival (PFS; not reached vs. 16.0 months; p < 0.001) and overall survival (OS; not reached vs. 41.0 months; p < 0.001) as compared with those in the dFLC ≥ 50 mg/L group. Furthermore, we confirmed that achieving complete response (CR) or low dFLC partial response (PR) predicted better OS in patients with initial dFLC ≥ 20 mg/L (not reached vs. 19 months; p = 0.005). Patients with initial dFLC < 50 mg/L represented distinct clinical manifestations and outcomes. Achieving CR or low dFLC PR might represent potential therapy goals allowing better survival and organ response in patients with dFLC between 20 and 50 mg/L.


Assuntos
Cadeias Leves de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida
17.
Clin Lymphoma Myeloma Leuk ; 18(6): 408-414, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29625928

RESUMO

BACKGROUND: AL amyloidosis might increase the risk of thromboembolism and other plasma cell dyscrasias; however, only a few reports have described the clinical features of thromboembolism. The present study aimed to elucidate the clinical features of thromboembolic events and to identify the risk factors for these events. MATERIALS AND METHODS: The medical records were retrospectively reviewed to define the clinically significant thromboembolic events. RESULTS: A total of 106 patients with biopsy-proven AL amyloidosis were included. During a median follow-up of 18.1 months (range, 0.4-166.9 months), 13 thromboembolism events were identified in 13 patients. Of the 13 patients, 9 (8.5%) experienced acute cerebral infarction, 2 (1.9%) experienced pulmonary embolism, and 2 (1.9%) experienced deep vein thrombosis. Patients with a higher serum free light chain (FLC) difference (≥ 172.4 mg/L) or ß2-microglobulin (ß2MG) levels (≥ 2.78 mg/L) experienced significantly more thromboembolic events compared with those with a lower value according to multivariable analysis (for FLC difference: hazard ratio, 4.309; 95% confidence interval, 1.158-16.032; P = .029; for ß2MG: hazard ratio, 9.739; 95% confidence interval, 1.127-84.174; P = .039). Most thromboembolic events (11 of 13; 84.6%) occurred within the first year after the AL amyloidosis diagnosis. CONCLUSION: The incidence of thromboembolism was substantial in those with AL amyloidosis. A greater FLC difference and for ß2MG levels were risk factors for thromboembolic events.


Assuntos
Cadeias Leves de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Tromboembolia/epidemiologia , Microglobulina beta-2/sangue , Adulto , Idoso , Biópsia , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Tromboembolia/sangue , Tromboembolia/etiologia
18.
Blood ; 131(14): 1568-1575, 2018 04 05.
Artigo em Inglês | MEDLINE | ID: mdl-29386197

RESUMO

Growth differentiation factor-15 (GDF-15) improves prognostication in patients with cardiovascular disorders in addition to conventional cardiac markers (N-terminal pro B-type natriuretic peptide [NT-proBNP], troponins [Tns]) and has shown prognostic value in patients with renal diseases. In patients with light chain (AL) amyloidosis, cardiac involvement is the major determinant of prognosis, and cardiac markers define prognosis, whereas biomarkers of renal involvement stratify renal risk. We explored the prognostic importance of serum level of GDF-15 in patients with AL amyloidosis in 2 independent cohorts. The prognostic value of GDF-15 level was initially evaluated in a cohort of 107 consecutive previously untreated patients with AL amyloidosis from Athens, Greece, and was then validated in a second cohort of 202 consecutive previously untreated patients from Pavia, Italy. High GDF-15 level was associated with a higher risk of early death and poor overall survival independently of NT-proBNP and high-sensitivity TnT (hsTnT) or hsTnI levels. At the 6-month landmark, reduction of GDF-15 level ≥25% was associated with improved outcome. GDF-15 level ≥4000 pg/mL was associated with a high risk of progression to dialysis, independently of renal risk defined by estimated glomerular filtration rate and proteinuria, in both cohorts; failure to reduce GDF-15 below this level was associated with increased risk at either the 3- or 6-month landmark, independently of the established renal response or progression criteria. In conclusion, GDF-15 has prognostic implications for different outcomes in patients with AL and adds prognostic information independent of that provided by cardiac and renal risk biomarkers.


Assuntos
Fator 15 de Diferenciação de Crescimento/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Rim/metabolismo , Idoso , Biomarcadores/sangue , Intervalo Livre de Doença , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/terapia , Masculino , Pessoa de Meia-Idade , Terapia de Substituição Renal , Taxa de Sobrevida
19.
Leukemia ; 32(6): 1421-1426, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29483709

RESUMO

We evaluated the prognostic impact of clonal circulating plasma cells (cPCs) detected by six-color multi-parametric flow cytometry (MFC) in light chain (AL) amyloidosis at diagnosis. Of the 154 patients who underwent MFC, cPCs were detected in 42% (n = 65) patients. Median number of cPCs was 81 per 150,000 events (range: 6-17,844). High bone marrow plasma cell percentage was an independent predictor of presence of cPCs. Presence of cPCs at diagnosis was associated with inferior overall survival (OS) (90 vs. 98 months, p = 0.003) and inferior progression free survival (PFS) (31 vs. 52 months, p = 0.02). Estimated 1, 2 and 5 year OS in the two groups was: 74, 64 and 57 and 89, 87, and 80%, respectively. Estimated PFS at 1, 2, and 5 years was: 69, 56, and 23% and 80, 74, and 37%, respectively. Furthermore, the presence of cPCs at diagnosis was an independent adverse predictor of OS in multivariable analysis. Achieving a very-good partial response, or better, was able to overcome the adverse impact of cPCs at diagnosis. Patients with cPCs at diagnosis may warrant closer monitoring post-treatment, especially if they do not achieve a deep hematologic response.


Assuntos
Citometria de Fluxo/métodos , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Plasmócitos , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Masculino , Pessoa de Meia-Idade , Prognóstico
20.
Expert Rev Hematol ; 11(2): 117-127, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29307226

RESUMO

INTRODUCTION: The most common form of systemic amyloidosis in Western countries is light chain amyloidosis. It is characterized by the deposition of a misfolded light chain in target organs. This amyloid precursor is produced by a usually small but dangerous B-cell clone. Areas covered: This review examines the diagnostic workup of this disease and current knowledge of biomarker-based staging systems. In addition, a risk-adapted treatment approach is presented, as well as an overview of the new treatment strategies. Expert commentary: The cornerstone of treatment is rapid and effective chemotherapy targeting the underlying plasma cell clone. In the near future, this will probably be associated with novel approaches targeting other steps of the amyloid cascade that result in amyloid deposits. Currently available effective treatments can alter its natural history if an early diagnosis is made. The availability of novel, more powerful drugs, and identification of the cellular mechanisms of organ damage and of the characteristics of the amyloidogenic plasma-cell clone all give grounds to hope that a dramatic improvement in the treatment of this disease will be seen in the near future.


Assuntos
Linfócitos B/metabolismo , Amiloidose de Cadeia Leve de Imunoglobulina , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Amiloidose de Cadeia Leve de Imunoglobulina/terapia
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