Evidence map›Paper›PMID 36697388›Full record

Observational studyBlood cancer journal2023

The management of light chain (AL) amyloidosis in Europe: clinical characteristics, treatment patterns, and efficacy outcomes between 2004 and 2018.

Giovanni Palladini, Stefan Schönland, Giampaolo Merlini, Paolo Milani, Arnaud Jaccard, Frank Bridoux, Meletios A Dimopoulos, Sriram Ravichandran, Ute Hegenbart, Wilfried Roeloffzen and 11 more

Abstract readObservational Study
In one paragraph

Observational study in Blood cancer journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
32citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

32 citing papers in PubMed, 1 synthesis or guideline pooled it.

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  14. Current cardiology reports · 2025
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors.

Giovanni PalladiniAmyloidosis Research and Treatment Center, Foundation "Instituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo", Department of Molecular Medicine, University of Pavia, Pavia, Italy.ORCID 0000-0001-5994-5138
Stefan SchönlandMedical Department V, Amyloidosis Center Heidelberg, University of Heidelberg, Heidelberg, Germany.ORCID 0000-0002-4853-5579
Giampaolo MerliniAmyloidosis Research and Treatment Center, Foundation "Instituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo", Department of Molecular Medicine, University of Pavia, Pavia, Italy.ORCID 0000-0001-7680-3254
Paolo MilaniAmyloidosis Research and Treatment Center, Foundation "Instituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo", Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Arnaud JaccardNational Amyloidosis Center and Hematology Unit, CHU Limoges, Limoges, France.ORCID 0000-0001-7091-8253
Frank BridouxNephrology Unit, CHU Poitiers, Poitiers, France.
Meletios A DimopoulosDepartment of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.ORCID 0000-0001-8990-3254
Sriram RavichandranNational Amyloidosis Centre, University College London, London, UK.
Ute HegenbartMedical Department V, Amyloidosis Center Heidelberg, University of Heidelberg, Heidelberg, Germany.ORCID 0000-0003-1917-6746
Wilfried RoeloffzenAmyloidosis Centre of Expertise Department of Internal Medicine, Faculty of Medical Sciences, University Medical Center Groningen, Groningen, Netherlands.
M Teresa CibeiraAmyloidosis and Myeloma Unit, Department of Hematology, Hospital Clinic de Barcelona, IDIBAPS, Barcelona, Spain.
Hermine AgisDepartment of Internal Medicine I, Division of Oncology, Medical University Vienna, Vienna, Austria.
Monique C MinnemaDepartment of Hematology, University Medical Center Utrecht, Utrecht, Netherlands.ORCID 0000-0002-3139-8379
Rui BergantimClinical Hematology, Centro Hospitalar São João, Porto, Portugal; i3S - Instituto de Investigação e Inovação em Saúde, University of Porto, Porto, Portugal; Cancer Drug Resistance Group, IPATIMUP - Institute of Molecular Pathology and Immunology of the University of Porto, Porto, Portugal; Clinical Hematology, FMUP - Faculty of Medicine, University of Porto, Porto, Portugal.
Roman HájekDepartment of Haematooncology, University Hospital Ostrava, and Department of Haematooncology, Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic.ORCID 0000-0001-6955-6267
Cristina JoãoHematology Department, Champalimaud Center for the Unknown, Lisbon, Portugal.ORCID 0000-0002-3978-766X
Alexandros LeonidakisHealth Data Specialists, Dublin, Ireland.
Giorgos CheliotisHealth Data Specialists, Dublin, Ireland.
Pieter SonneveldErasmus MC Cancer Institute, Rotterdam, Netherlands.ORCID 0000-0002-0808-2237
Efstathios KastritisDepartment of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece. ekastritis@gmail.com.ORCID 0000-0001-8191-5832
Ashutosh WechalekarNational Amyloidosis Centre, University College London, London, UK.ORCID 0000-0002-4406-7237

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Systemic light-chain (AL) amyloidosis is a rare and debilitating disease. Advances have been made in new treatments in recent years, yet real-world data on the management of the disease are scarce. EMN23 is a retrospective, observational study of patients who initiated first-line treatment in 2004-2018 in Europe, presenting the demographics, clinical characteristics, treatment patterns, and outcomes, from 4480 patients. Regimens based on bortezomib were the most frequently used as first-line therapy; only 6.2% of the patients received autologous stem cell transplant. Hematologic responses improved post-2010 (67.1% vs 55.6% pre-2010). The median overall survival (OS) was 48.8 (45.2-51.7) months; 51.4 (47.3-57.7) months pre-2010 and 46.7 (41.3-52.2) months post-2010. Early mortality was 13.4% and did not improve (11.4% vs 14.4% pre- and post-2010); furthermore, it remained high in patients with advanced cardiac disease (over 39% for stage IIIb). There was a significant improvement for stage IIIa (14.2 vs 30.7 months, p = 0.0170) but no improvement for stage IIIb patients (5.0 vs 4.5 months). This European real-world study of AL-amyloidosis emphasizes the unmet needs of early diagnosis, and the lack of improvement in survival outcomes of the frail stage IIIb population, despite the introduction of new therapies in recent years.

Indexed as

Immunoglobulin Light-chain AmyloidosisBortezomibEuropeHumansRetrospective StudiesStem Cell TransplantationTreatment OutcomeBortezomib

Identifiers

PMID36697388
PMCPMC9876983

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.