Nutritional status independently affects quality of life of patients with systemic immunoglobulin light-chain (AL) amyloidosis

Background: Immunoglobulin light chain (AL) amyloidosis is a complex disease marked by a poor clinical portrait and prognosis generally leading to organ dysfunction and shortened survival. We aimed to review the available evidence on whether AL amyloidosis can lead to malnutrition, thus having a negative impact on quality of life (QoL) and survival. Materials: We searched Pubmed with no restrictions to the year of publication or language. Retrospective or prospective, observational, and interventional studies that reported data regarding AL amyloidosis and nutritional status were included. Results: From 62 articles retrieved, 23 were included. Malnutrition was prevalent in up to 65% of patients with AL Amyloidosis. Prevalence of weight loss of 10% or more ranged from 6 to 22% of patients, while a body mass index of < 22 kg/m2 was found in 22 to 42%. Weight loss, lower BMI and other indicators of poor nutritional status were negatively associated with quality of life and survival. Only one RCT focused on nutritional counselling was found and reported positive results on patients' QoL and survival. Conclusion Despite inconsistencies across assessment criteria, the available data reveal that weight loss and malnutrition are common features in patients with AL amyloidosis. This review reinforces the premise that an impaired nutritional status can be negatively associated with QoL and survival in patients with AL amyloidosis, and therefore should be further investigated.

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Nutritional counseling improves quality of life and preserves body weight in systemic immunoglobulin light-chain (AL) amyloidosis

Nutrition ◽

10.1016/j.nut.2015.04.011 ◽

2015 ◽

Vol 31 (10) ◽

pp. 1228-1234 ◽

Cited By ~ 15

Author(s):

Riccardo Caccialanza ◽

Giovanni Palladini ◽

Emanuele Cereda ◽

Chiara Bonardi ◽

Paolo Milani ◽

...

Keyword(s):

Quality Of Life ◽

Body Weight ◽

Light Chain ◽

Al Amyloidosis ◽

Immunoglobulin Light Chain ◽

Nutritional Counseling

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OP038 NUTRITIONAL STATUS AND QUALITY OF LIFE IN OUTPATIENTS WITH SYSTEMIC IMMUNOGLOBULIN LIGHT-CHAIN AMYLOIDOSIS (AL) AT DIAGNOSIS

Clinical Nutrition Supplements ◽

10.1016/s1744-1161(10)70064-8 ◽

2010 ◽

Vol 5 (2) ◽

pp. 16 ◽

Cited By ~ 1

Author(s):

R. Caccialanza ◽

G. Palladini ◽

C. Klersy ◽

C. Bonardi ◽

B. Cameletti ◽

...

Keyword(s):

Quality Of Life ◽

Nutritional Status ◽

Light Chain ◽

Immunoglobulin Light Chain ◽

Light Chain Amyloidosis ◽

Immunoglobulin Light Chain Amyloidosis

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Amyloidosis: diagnosis and new therapies for a misunderstood and misdiagnosed disease

Neurodegenerative Disease Management ◽

10.2217/nmt-2019-0020 ◽

2019 ◽

Vol 9 (6) ◽

Cited By ~ 2

Author(s):

Victoria E Thomas ◽

Justin Smith ◽

Merrill D Benson ◽

Noel R Dasgupta

Keyword(s):

Quality Of Life ◽

Light Chain ◽

Protein Identification ◽

Amyloid Fibril ◽

Immunoglobulin Light Chain ◽

Transthyretin Amyloidosis ◽

Identification Methods ◽

Novel Treatments ◽

Noninvasive Cardiac Imaging

Amyloidosis is a group of diseases characterized by extracellular deposition of amyloid fibril complexes. Fibril deposition results in organ dysfunction and possible failure. Amyloidosis is regarded as a rare disease, but in general is underdiagnosed. The two main types of systemic amyloidosis are immunoglobulin light chain and transthyretin amyloidosis. The increased availability of noninvasive cardiac imaging, genetic testing and improved laboratory assays and protein identification methods have led to increased diagnosis. However, in many cases, the diagnosis is not made until the patient develops organ impairment. Earlier diagnosis is required to prevent irreversible organ failure. Novel treatments for immunoglobulin light chain and transthyretin amyloidosis that halt disease progression, prolong and increase quality of life have recently become available.

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Associations between Health-Related Quality of Life and Self-Reported Emergency Room Department Visits and Inpatient Hospitalizations: Insights from a Secondary Data Analysis of Patients with Light-Chain (AL) Amyloidosis

PharmacoEconomics - Open ◽

10.1007/s41669-019-0122-7 ◽

2019 ◽

Vol 3 (3) ◽

pp. 367-375

Author(s):

Kristen L. McCausland ◽

Avery A. Rizio ◽

Michelle K. White ◽

Martha S. Bayliss ◽

Tiffany P. Quock

Keyword(s):

Quality Of Life ◽

Data Analysis ◽

Light Chain ◽

Secondary Data ◽

Al Amyloidosis ◽

Health Related Quality ◽

Inpatient Hospitalizations ◽

Related Quality ◽

Health Related

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Response of factor X deficiency to darutumumab in the treatment of AL amyloidosis: a novel finding

BMJ Case Reports ◽

10.1136/bcr-2020-240631 ◽

2021 ◽

Vol 14 (4) ◽

pp. e240631

Author(s):

Ethan Mar ◽

Kerry Taylor ◽

Peter Mollee

Keyword(s):

Quality Of Life ◽

Case Report ◽

Light Chain ◽

Al Amyloidosis ◽

Factor X ◽

Haematological Response ◽

Factor X Deficiency ◽

First Case ◽

Complete Haematological Response

We report a case of progressive light-chain amyloidosis (otherwise known as AL amyloidosis) with acquired factor X (aFX) deficiency with a complete haematological response and rapid normalisation of FX levels following daratumumab monotherapy. To our knowledge, this is the first case report documenting successful treatment with daratumumab of aFX deficiency secondary to AL amyloidosis. The patient responded well to this therapy, with excellent symptomatic and quality of life improvements as well as a reduction in bleeding manifestations. This case highlights the value in considering daratumumab treatment when AL amyloidosis is complicated by FX deficiency.

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Case Report: Management of Primary Tracheobronchial Light Chain Amyloidosis in a Patient With Biclonal Gammopathy Using a Systemic Bortezomib-Based Regimen

Frontiers in Medicine ◽

10.3389/fmed.2021.728561 ◽

2021 ◽

Vol 8 ◽

Author(s):

Wei Yan ◽

Peng Li ◽

Cen Wu ◽

Chuming Zhou ◽

Aijun Liao ◽

...

Keyword(s):

Quality Of Life ◽

Light Chain ◽

Local Treatment ◽

Wall Thickening ◽

Current View ◽

Al Amyloidosis ◽

Airway Patency ◽

Biclonal Gammopathy ◽

Localized Form

Primary tracheobronchial light chain (AL) amyloidosis is a rare and heterogeneous disease characterized by the buildup of amyloid deposits in the airway mucosa. Although its treatment remains challenging, the current view is that the localized form can be treated conservatively due to its slow progression. While radiotherapy has proven effective in treating localized form of the disease, some patients do not respond to local treatment and continue to experience poor quality of life, highlighting the need to explore additional treatment strategies. In this report, we discuss a case of primary tracheobronchial AL amyloidosis with biclonal gammopathy (IgA κ and IgG κ) in a 46-year-old man who was transferred to our hospital due to dyspnea progression over the preceding 3 years. Chest computed tomography revealed irregular tracheobronchial stenosis with wall thickening, and histological examination of the bronchial biopsies confirmed the diagnosis of endobronchial AL amyloidosis. Owing to the poor effect of radiation therapy and treatments for improving airway patency, he was treated with a systemic chemotherapy regimen [cyclophosphamide-bortezomib-dexamethasone (CyBorD)]. We observed substantial improvements in his dyspnea, highlighting the potential of systemic therapy to improve quality of life of patients with tracheobronchial AL amyloidosis. However, the long-term pathological changes associated with local bronchial lesions require further investigation.

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