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European Collaborative Study Defining Clinical Profile Outcomes and Novel Prognostic Criteria in Monoclonal Immunoglobulin M-Related Light Chain Amyloidosis.

Identifieur interne : 001D25 ( PubMed/Corpus ); précédent : 001D24; suivant : 001D26

European Collaborative Study Defining Clinical Profile Outcomes and Novel Prognostic Criteria in Monoclonal Immunoglobulin M-Related Light Chain Amyloidosis.

Auteurs : Sajitha Sachchithanantham ; Murielle Roussel ; Giovanni Palladini ; Catherine Klersy ; Shameem Mahmood ; Christopher Paul Venner ; Simon Gibbs ; Julian Gillmore ; Helen Lachmann ; Philip N. Hawkins ; Arnaud Jaccard ; Giampaolo Merlini ; Ashutosh D. Wechalekar

Source :

RBID : pubmed:27114592

English descriptors

Abstract

Immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 6% to 10% of all AL amyloidosis cases, is a rare and poorly studied clinical entity. Its natural history and management is not clearly defined. Prognostic and response criteria for AL amyloidosis in general have not been validated in this population.

DOI: 10.1200/JCO.2015.63.3123
PubMed: 27114592

Links to Exploration step

pubmed:27114592

Le document en format XML

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<term>Humans</term>
<term>Immunoglobulin Light Chains (immunology)</term>
<term>Immunoglobulin M (immunology)</term>
<term>Lymphoma, Non-Hodgkin (drug therapy)</term>
<term>Lymphoma, Non-Hodgkin (immunology)</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Prognosis</term>
<term>Retrospective Studies</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en">
<term>Immunoglobulin Light Chains</term>
<term>Immunoglobulin M</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en">
<term>Amyloidosis</term>
</keywords>
<keywords scheme="MESH" qualifier="drug therapy" xml:lang="en">
<term>Amyloidosis</term>
<term>Lymphoma, Non-Hodgkin</term>
</keywords>
<keywords scheme="MESH" qualifier="immunology" xml:lang="en">
<term>Amyloidosis</term>
<term>Lymphoma, Non-Hodgkin</term>
</keywords>
<keywords scheme="MESH" qualifier="therapeutic use" xml:lang="en">
<term>Antineoplastic Combined Chemotherapy Protocols</term>
</keywords>
<keywords scheme="MESH" qualifier="therapy" xml:lang="en">
<term>Amyloidosis</term>
</keywords>
<keywords scheme="MESH" xml:lang="en">
<term>Aged</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Prognosis</term>
<term>Retrospective Studies</term>
</keywords>
</textClass>
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<front>
<div type="abstract" xml:lang="en">Immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 6% to 10% of all AL amyloidosis cases, is a rare and poorly studied clinical entity. Its natural history and management is not clearly defined. Prognostic and response criteria for AL amyloidosis in general have not been validated in this population.</div>
</front>
</TEI>
<pubmed>
<MedlineCitation Status="MEDLINE" Owner="NLM">
<PMID Version="1">27114592</PMID>
<DateCreated>
<Year>2016</Year>
<Month>06</Month>
<Day>02</Day>
</DateCreated>
<DateCompleted>
<Year>2017</Year>
<Month>06</Month>
<Day>30</Day>
</DateCompleted>
<DateRevised>
<Year>2017</Year>
<Month>06</Month>
<Day>30</Day>
</DateRevised>
<Article PubModel="Print-Electronic">
<Journal>
<ISSN IssnType="Electronic">1527-7755</ISSN>
<JournalIssue CitedMedium="Internet">
<Volume>34</Volume>
<Issue>17</Issue>
<PubDate>
<Year>2016</Year>
<Month>Jun</Month>
<Day>10</Day>
</PubDate>
</JournalIssue>
<Title>Journal of clinical oncology : official journal of the American Society of Clinical Oncology</Title>
<ISOAbbreviation>J. Clin. Oncol.</ISOAbbreviation>
</Journal>
<ArticleTitle>European Collaborative Study Defining Clinical Profile Outcomes and Novel Prognostic Criteria in Monoclonal Immunoglobulin M-Related Light Chain Amyloidosis.</ArticleTitle>
<Pagination>
<MedlinePgn>2037-45</MedlinePgn>
</Pagination>
<ELocationID EIdType="doi" ValidYN="Y">10.1200/JCO.2015.63.3123</ELocationID>
<Abstract>
<AbstractText Label="PURPOSE" NlmCategory="OBJECTIVE">Immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 6% to 10% of all AL amyloidosis cases, is a rare and poorly studied clinical entity. Its natural history and management is not clearly defined. Prognostic and response criteria for AL amyloidosis in general have not been validated in this population.</AbstractText>
<AbstractText Label="PATIENTS AND METHODS" NlmCategory="METHODS">We retrospectively gathered data for 250 patients diagnosed with IgM AL amyloidosis from three European amyloidosis centers. Clinical features, hematologic response, and overall survival (OS) were analyzed. The current staging and response criteria in non-IgM AL amyloidosis was applied to this series to assess its utility in this patient cohort.</AbstractText>
<AbstractText Label="RESULTS" NlmCategory="RESULTS">Patients with IgM AL amyloidosis have a significant IgM paraprotein (median, 10 g/L), less frequent lambda light chain isotype, and evaluable difference between involved and uninvolved free light chains (dFLCs; > 50 mg/L) in only two thirds of patients. Bone marrow showed clear non-Hodgkin lymphoma as the underlying disorder in 54% of patients. Cardiac involvement (45%) is less common but there is more frequent lymph node (20%) and neuropathic (28%) involvement compared with non-IgM AL. Fifty-seven percent of patients achieved a hematologic response (14% very good partial response/complete response [VGPR/CR]), with median OS not reached for patients achieving VGPR/CR, 64 months for PR, and 28 months for nonresponders (P < .001). On multivariate analysis, cardiac involvement, advanced Mayo disease stage, neuropathic involvement, and liver involvement were independent factors that had an impact on survival. Combining abnormal N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin T with liver involvement and the presence of neuropathy gives a better risk model: median OS of patients with none, one, or two or more abnormal factors was 90, 33, and 16 months, respectively.</AbstractText>
<AbstractText Label="CONCLUSION" NlmCategory="CONCLUSIONS">IgM AL amyloidosis is a distinct clinical entity. Low-risk disease can be defined by combining cardiac involvement with novel prognostic markers. Deeper hematologic responses translate into improved outcomes, yet deep responses remain dismally poor, which highlights the urgent need for novel therapies.</AbstractText>
<CopyrightInformation>© 2016 by American Society of Clinical Oncology.</CopyrightInformation>
</Abstract>
<AuthorList CompleteYN="Y">
<Author ValidYN="Y">
<LastName>Sachchithanantham</LastName>
<ForeName>Sajitha</ForeName>
<Initials>S</Initials>
<AffiliationInfo>
<Affiliation>Sajitha Sachchithanantham, Murielle Roussel, Shameem Mahmood, Julian Gillmore, Helen Lachmann, Philip N. Hawkins, and Ashutosh D. Wechalekar, University College London Medical School, London, United Kingdom; Murielle Roussel, Centre Hospitalier Universitaire (CHU) Purpan, Toulouse; Arnaud Jaccard, CHU Limoges, Limoges, France; Giovanni Palladini and Giampaolo Merlini, University of Pavia; Catherine Klersy, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Christopher Paul Venner, University of Alberta, Edmonton, Alberta, Canada; and Simon Gibbs, Westmead Hospital, Sydney, New South Wales, Australia.</Affiliation>
</AffiliationInfo>
</Author>
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<Affiliation>Sajitha Sachchithanantham, Murielle Roussel, Shameem Mahmood, Julian Gillmore, Helen Lachmann, Philip N. Hawkins, and Ashutosh D. Wechalekar, University College London Medical School, London, United Kingdom; Murielle Roussel, Centre Hospitalier Universitaire (CHU) Purpan, Toulouse; Arnaud Jaccard, CHU Limoges, Limoges, France; Giovanni Palladini and Giampaolo Merlini, University of Pavia; Catherine Klersy, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Christopher Paul Venner, University of Alberta, Edmonton, Alberta, Canada; and Simon Gibbs, Westmead Hospital, Sydney, New South Wales, Australia.</Affiliation>
</AffiliationInfo>
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<Affiliation>Sajitha Sachchithanantham, Murielle Roussel, Shameem Mahmood, Julian Gillmore, Helen Lachmann, Philip N. Hawkins, and Ashutosh D. Wechalekar, University College London Medical School, London, United Kingdom; Murielle Roussel, Centre Hospitalier Universitaire (CHU) Purpan, Toulouse; Arnaud Jaccard, CHU Limoges, Limoges, France; Giovanni Palladini and Giampaolo Merlini, University of Pavia; Catherine Klersy, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Christopher Paul Venner, University of Alberta, Edmonton, Alberta, Canada; and Simon Gibbs, Westmead Hospital, Sydney, New South Wales, Australia.</Affiliation>
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<ForeName>Catherine</ForeName>
<Initials>C</Initials>
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<Affiliation>Sajitha Sachchithanantham, Murielle Roussel, Shameem Mahmood, Julian Gillmore, Helen Lachmann, Philip N. Hawkins, and Ashutosh D. Wechalekar, University College London Medical School, London, United Kingdom; Murielle Roussel, Centre Hospitalier Universitaire (CHU) Purpan, Toulouse; Arnaud Jaccard, CHU Limoges, Limoges, France; Giovanni Palladini and Giampaolo Merlini, University of Pavia; Catherine Klersy, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Christopher Paul Venner, University of Alberta, Edmonton, Alberta, Canada; and Simon Gibbs, Westmead Hospital, Sydney, New South Wales, Australia.</Affiliation>
</AffiliationInfo>
</Author>
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<ForeName>Shameem</ForeName>
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</AffiliationInfo>
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<LastName>Wechalekar</LastName>
<ForeName>Ashutosh D</ForeName>
<Initials>AD</Initials>
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<Affiliation>Sajitha Sachchithanantham, Murielle Roussel, Shameem Mahmood, Julian Gillmore, Helen Lachmann, Philip N. Hawkins, and Ashutosh D. Wechalekar, University College London Medical School, London, United Kingdom; Murielle Roussel, Centre Hospitalier Universitaire (CHU) Purpan, Toulouse; Arnaud Jaccard, CHU Limoges, Limoges, France; Giovanni Palladini and Giampaolo Merlini, University of Pavia; Catherine Klersy, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Christopher Paul Venner, University of Alberta, Edmonton, Alberta, Canada; and Simon Gibbs, Westmead Hospital, Sydney, New South Wales, Australia. a.wechalekar@ucl.ac.uk.</Affiliation>
</AffiliationInfo>
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<Language>eng</Language>
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<PublicationType UI="D016448">Multicenter Study</PublicationType>
</PublicationTypeList>
<ArticleDate DateType="Electronic">
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<Month>04</Month>
<Day>25</Day>
</ArticleDate>
</Article>
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<NlmUniqueID>8309333</NlmUniqueID>
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</MeshHeading>
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<QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName>
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<QualifierName UI="Q000276" MajorTopicYN="N">immunology</QualifierName>
<QualifierName UI="Q000628" MajorTopicYN="Y">therapy</QualifierName>
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</MeshHeading>
<MeshHeading>
<DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName>
</MeshHeading>
<MeshHeading>
<DescriptorName UI="D007147" MajorTopicYN="N">Immunoglobulin Light Chains</DescriptorName>
<QualifierName UI="Q000276" MajorTopicYN="N">immunology</QualifierName>
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<DescriptorName UI="D007075" MajorTopicYN="N">Immunoglobulin M</DescriptorName>
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<MeshHeading>
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<QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName>
<QualifierName UI="Q000276" MajorTopicYN="N">immunology</QualifierName>
</MeshHeading>
<MeshHeading>
<DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName>
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<Day>27</Day>
<Hour>6</Hour>
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<Month>7</Month>
<Day>1</Day>
<Hour>6</Hour>
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