Advertisement

Topics

Ten-year survivors in AL amyloidosis: characteristics and treatment pattern.

08:00 EDT 12th July 2019 | BioPortfolio

Summary of "Ten-year survivors in AL amyloidosis: characteristics and treatment pattern."

Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long-term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten-year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10-year survivors compared to those who did not survive to 10 years. All-time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first-line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty-seven percent of the 10-year survivors did not require a second-line therapy. The median treatment-free survival (TFS) among the 10-year survivors was 10·5 years (interquartile range 7·4-12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long-term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.

Affiliation

Journal Details

This article was published in the following journal.

Name: British journal of haematology
ISSN: 1365-2141
Pages:

Links

DeepDyve research library

PubMed Articles [30100 Associated PubMed Articles listed on BioPortfolio]

Problems of diagnostics and treatment of transtiretinum amyloidosis with destruction of the heart in the elderly: Clinical experience.

The article presents a modern understanding of the treatment of cardiac amyloidosis, provides data on the frequency of occurrence, classification and variants of clinical manifestations of transthyret...

Prevalence and clinical implications of t(11;14) in patients with amyloid light-chain amyloidosis with or without concurrent multiple myeloma.

According to fluorescent in situ hybridization, t(11;14) is the most common cytogenetic abnormality in amyloid light-chain (AL) amyloidosis, but its prevalence in patients with AL amyloidosis and conc...

A Case of Localized Amyloidosis of the Right Ureter Treated Effectively by Occulusive Dressing Technique Therapy Using Dimethyl Sulfoxide : Long-Term Follow-Up of Eight Years.

The patient was a 56-year-old female. She was referred to our department for further examination of right hydronephrosis in 2010. Computed tomography (CT) showed right hydronephrosis, and retrograde p...

15 Year Overall Survival Rates after Autologous Stem Cell Transplantation for AL Amyloidosis.

In appropriately selected patients with AL amyloidosis, autologous stem cell transplant (ASCT) is an established treatment modality with excellent outcomes and decreasing transplant related mortality ...

Risk of subsequent primary leukaemias among 69,460 five-year survivors of childhood cancer diagnosed from 1940 to 2008 in Europe: A cohort study within PanCareSurFup.

Survivors of childhood cancers are at risk of developing subsequent primary leukaemias (SPLs), but the long-term risks beyond 20 years of treatment are still unclear. We investigated the risk of SPLs ...

Clinical Trials [7503 Associated Clinical Trials listed on BioPortfolio]

Characteristics of Patients With Amyloidosis & Heart Failure Being Evaluated for a Heart Transplant

The purpose of this study is to describe the characteristics of patients with amyloidosis and severe heart failure being evaluated for cardiac and stem cell transplantation.

HELIOS-A: A Study of ALN-TTRSC02 in Patients With Hereditary Transthyretin Amyloidosis (hATTR Amyloidosis)

The purpose of this study is to evaluate the efficacy and safety of ALN-TTRSC02 in patients with hereditary transthyretin amyloidosis (hATTR amyloidosis). Participants will receive ALN-TTR...

Monitoring of Early Disease Progression in Hereditary Transthyretin Amyloidosis

This study measures circulating, misfolded ATTR oligomers in asymptomatic ATTRm amyloidosis genetic carriers longitudinally over five years.

Daratumumab for the Treatment of Patients With AL Amyloidosis

Participants with AL Amyloidosis will receive the drug daratumumab by IV infusion once weekly for two months, then every 2 weeks for four months, then once each month. Study treatment may ...

Intensive Versus Conventional Treatment in Patients With Primary Amyloidosis

AL amyloidosis is caused by a clonal plasma cell dyscrasia and characterized by progressive deposition of amyloid fibrils derived from monoclonal Ig light chains, leading to multisystem or...

Medical and Biotech [MESH] Definitions

Diseases in which there is a familial pattern of AMYLOIDOSIS.

El Nino-Southern Oscillation or ENSO is a cycle of extreme alternating warm El Niño and cold La Nina events which is the dominant year-to-year climate pattern on Earth. Both terms refer to large-scale changes in sea-surface temperature across the eastern tropical Pacific. ENSO is associated with a heightened risk of certain vector-borne diseases. (From http://www.elnino.noaa.gov/lanina_new_faq.html, accessed 5/12/2020)

Those characteristics that distinguish one SEX from the other. The primary sex characteristics are the OVARIES and TESTES and their related hormones. Secondary sex characteristics are those which are masculine or feminine but not directly related to reproduction.

The personality pattern or syndrome consisting of behavioral and attitudinal characteristics reflecting a preoccupation with the factors of power and authority in interpersonal relationships.

Differences in measurable biological values, characteristics, or traits, within one individual under different conditions for the individual such as fasting, season of the year, age, or state of wellness.

Advertisement
Quick Search
Advertisement
Advertisement

 


DeepDyve research library

Relevant Topic

Blood
Blood is a specialized bodily fluid that delivers necessary substances to the body's cells (in animals) – such as nutrients and oxygen – and transports waste products away from those same cells.  In vertebrates, it is composed of blo...


Searches Linking to this Article