Disease burden of systemic light-chain amyloidosis: a systematic literature review

  title={Disease burden of systemic light-chain amyloidosis: a systematic literature review},
  author={Huamao Mark Lin and Xin Gao and Catherine E. Cooke and Deborah T. Berg and Richard Labotka and Douglas V. Faller and Brian Seal and Parameswaran N. Hari},
  journal={Current Medical Research and Opinion},
  pages={1017 - 1031}
  • H. Lin, Xin Gao, P. Hari
  • Published 11 April 2017
  • Medicine
  • Current Medical Research and Opinion
Abstract Introduction: A systematic literature review on systemic light chain (AL) amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps. Methods: MEDLINE, Embase and Cochrane databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic… 
Systematic literature review of evidence in amyloid light-chain amyloidosis.
A paucity of published literature relating to randomized controlled trials, utility values, economic evaluations and indirect costs in AL amyloidosis is highlighted.
Systemic Amyloidosis Recognition, Prognosis, and Therapy: A Systematic Review.
This review of the literature on systemic amyloidosis provides evidence-based recommendations that could allow clinicians to diagnose this rare set of diseases earlier and enable accurate staging and counseling about prognosis and concludes that all forms of amyloidsosis are underdiagnosed.
The Clinical Characteristics of Immunoglobulin Light Chain Amyloidosis in the Chinese Population: A Systematic Scoping Review (Preprint)
This scoping review suggests that most patients with AL amyloidosis were at an advanced stage when diagnosed in China, and Epidemiological studies with a larger sample size are needed to comprehensively investigate the AL ameloidosis patients’ characteristics in the Chinese population.
Epidemiology of AL amyloidosis: a real-world study using US claims data.
There was an increase in AL amyloidosis prevalence over a 9-year period coupled with stable incidence rates, and the number seems likely to rise, extrapolating from the data, there are at least 12 000 adults in the United States living with AL ameloidosis.
Treatment patterns and health care resource utilization among patients with relapsed/refractory systemic light chain amyloidosis
  • P. Hari, H. Lin, V. Sanchorawala
  • Medicine
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • 2018
RRAL amyloidosis is associated with high morbidity from target organ failure and mortality, which emphasizes the need for novel medications to improve care for patients with RRAL ameloidosis.
Untangling the clinical and economic burden of hospitalization for cardiac amyloidosis in the United States
Hospitalization for cardiac amyloidosis is costly, with high rates of readmission and mortality, and opportunities exist to improve care.
Beyond Survival in AL amyloidosis: Identifying and Satisfying Patients’ Needs
Supportive care requires an integrated, multidimensional and patient-centered approach to improve survival and feelings of well-being, as organ responses lag behind hematologic responses.
Healthcare resource utilization and costs in amyloid light-chain amyloidosis: a real-world study using US claims data.
Al chemotherapy-based prescribing practices changed and total annual healthcare costs increased over time among AL amyloidosis patients.


AL Amyloidosis: Development Of a Conceptual Model and a Symptom Diary
A comprehensive conceptual model of symptoms and impacts of disease and treatment was developed and could be used to identify important outcomes from the patients’ perspective that should be assessed in clinical trials to capture treatment benefit.
Clinical characteristics and outcome of primary systemic light-chain amyloidosis in Greece.
Heart involvement and bone marrow plasma cell infiltration comprise adverse prognostic factors but also indicate the heterogeneity of the disease and the need for individual treatment approaches.
Amyloidosis: pathogenesis and new therapeutic options.
  • G. Merlini, D. Seldin, M. Gertz
  • Medicine, Biology
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • 2011
Treatment for AL amyloidosis is highly individualized, determined on the basis of age, organ dysfunction, and regimen toxicities, and should be guided by biomarkers of hematologic and cardiac response.
Management of systemic AL amyloidosis: recommendations of the Myeloma Foundation of Australia Medical and Scientific Advisory Group
Improved appreciation of the need for risk stratification, standardised use of sensitive laboratory testing for monitoring disease response, vigilant supportive care and the availability of newer agents with more favourable toxicity profiles have contributed to the improvement in treatment‐related mortality and overall survival seen over the past decade.
Incidence of amyloidosis over 3 years: the AMYPRO study.
The clinician needs to be aware that in most of the amyloid cases identified on the pathologic examination there is no need for additional examination unless there are clinical or biological signs of a primary or secondary amyloidsosis.
Long Term Follow-up of Patients with Immunoglobulin Light Chain Amyloidosis Treated with Lenalidomide and Dexamethasone.
Lenalidomide in combination with dexamethasone can be associated with good progression free and overall survival rates, even among patients with relapsed or refractory disease, and is not recommended for patients with AL.
Light-chain (AL) amyloidosis: diagnosis and treatment.
  • V. Sanchorawala
  • Medicine, Biology
    Clinical journal of the American Society of Nephrology : CJASN
  • 2006
Approaches to diagnosis, assessment of disease severity, and treatment of AL amyloidosis are outlined, which are prolonging survival and preserving organ function in patients with this disease.
Re-examining the role of high-dose chemotherapy in the treatment of light chain amyloidosis.
  • S. Devine
  • Medicine, Biology
    Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation
  • 2014
Systemic light chain amyloidosis: an update for treating physicians.
In immunoglobulin light chain amyloidosis a small, indolent plasma cell clone synthesizes light chains that cause devastating organ damage and novel agents and therapeutic targets are expected to be exploited, in an integrated, more effective and less toxic treatment strategy.