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  1. Book: Systemic amyloidosis due to monoclonal immunoglobulins

    Comenzo, Raymond L.

    (Hematology/oncology clinics of North America ; volume 34, number 6 (December 2020))

    2020  

    Author's details editor Raymond L. Comenzo
    Series title Hematology/oncology clinics of North America ; volume 34, number 6 (December 2020)
    Hematology, oncology clinics of North America
    Collection Hematology, oncology clinics of North America
    Language English
    Size xvi Seiten, Seite 998-1214, Illustrationen
    Publisher Elsevier
    Publishing place Philadelphia, Pennsylvania
    Publishing country United States
    Document type Book
    HBZ-ID HT020671446
    ISBN 978-0-323-79165-6 ; 0-323-79165-4
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

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  2. Article ; Online: Commentary on No Monoclonal Protein in a Patient with CRAB Features.

    Comenzo, Raymond L

    Clinical chemistry

    2021  Volume 67, Issue 12, Page(s) 1588–1589

    Language English
    Publishing date 2021-12-01
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 80102-1
    ISSN 1530-8561 ; 0009-9147
    ISSN (online) 1530-8561
    ISSN 0009-9147
    DOI 10.1093/clinchem/hvab212
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Shedding Tears to Clear the Way.

    Comenzo, Raymond L

    Hematology/oncology clinics of North America

    2020  Volume 34, Issue 6, Page(s) xiii–xiv

    MeSH term(s) Humans ; Immunoglobulin Light-chain Amyloidosis ; Periodicals as Topic ; Portraits as Topic
    Language English
    Publishing date 2020-09-30
    Publishing country United States
    Document type Editorial ; Introductory Journal Article
    ZDB-ID 93115-9
    ISSN 1558-1977 ; 0889-8588
    ISSN (online) 1558-1977
    ISSN 0889-8588
    DOI 10.1016/j.hoc.2020.09.002
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  4. Article: Evaluating the Therapeutic Potential of Idecabtagene Vicleucel in the Treatment of Multiple Myeloma: Evidence to Date.

    Mann, Hashim / Comenzo, Raymond L

    OncoTargets and therapy

    2022  Volume 15, Page(s) 799–813

    Abstract: Over the past two decades, significant progress has been made in the diagnosis, risk assessment and treatment of patients with multiple myeloma, translating into remarkable improvements in survival outcomes. Yet, cure remains elusive, and almost all ... ...

    Abstract Over the past two decades, significant progress has been made in the diagnosis, risk assessment and treatment of patients with multiple myeloma, translating into remarkable improvements in survival outcomes. Yet, cure remains elusive, and almost all patients eventually experience relapse, particularly those with high-risk and refractory disease. Immune-based approaches have emerged as highly effective therapeutic options that have heralded a new era in the treatment of multiple myeloma. Idecabtagene vicleucel (ide-cel) is one such therapy that employs the use of genetically modified autologous T-cells to redirect immune activation in a tumor-directed fashion. It has yielded impressive responses even in patients with poor-risk disease and is the first chimeric antigen receptor (CAR) T-cell therapy to be approved for treatment in relapsed or refractory multiple myeloma. In this review, we examine the design and pharmacokinetics of ide-cel, audit evidence that led to its incorporation into the current treatment paradigm and provide insight into its clinical utilization with a focus on real-life intricacies.
    Language English
    Publishing date 2022-07-22
    Publishing country New Zealand
    Document type Journal Article ; Review
    ZDB-ID 2495130-4
    ISSN 1178-6930
    ISSN 1178-6930
    DOI 10.2147/OTT.S305429
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Immunotherapy in AL Amyloidosis.

    Zhang, Yifei / Comenzo, Raymond L

    Current treatment options in oncology

    2022  Volume 23, Issue 7, Page(s) 1059–1071

    Abstract: Opinion statement: Light-chain amyloidosis is a rare disorder where a small clone of plasma cells is producing excess toxic light chains that deposit in various organs and cause dysfunction. Cardiac involvement is a major determinant of survival and ... ...

    Abstract Opinion statement: Light-chain amyloidosis is a rare disorder where a small clone of plasma cells is producing excess toxic light chains that deposit in various organs and cause dysfunction. Cardiac involvement is a major determinant of survival and rapid reduction of light chain is critical for recovery of organ function and overall survival. Immunotherapy targeting the clonal plasma cells and amyloid fibrils has emerged as a promising candidate. Daratumumab, both alone and in combinations with other anti-myeloma agents, is able to achieve deep hematologic responses and has greatly improved outcomes. Isatuximab, elotuzumab, and CAEL101 have also shown promising results and further studies are ongoing in the frontline as well as the relapsed/refractory setting. The frailty of AL patients and the relapsing/remitting nature of the disease present unique challenges, and the low toxicity of monoclonal antibodies makes them well-suited for these patients. Other immunotherapy agents including chimeric antigen receptor T cells, bispecific antibodies, and antibody-drug conjugates have altered the landscape in treatment of multiple myeloma, and are in the early phase of evaluation in patients with AL amyloidosis with results eagerly awaited.
    MeSH term(s) Humans ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/etiology ; Immunoglobulin Light-chain Amyloidosis/therapy ; Immunologic Factors/therapeutic use ; Immunotherapy/adverse effects ; Immunotherapy/methods ; Multiple Myeloma/drug therapy ; Neoplasm Recurrence, Local
    Chemical Substances Immunologic Factors
    Language English
    Publishing date 2022-05-30
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, N.I.H., Extramural
    ZDB-ID 2057351-0
    ISSN 1534-6277 ; 1527-2729
    ISSN (online) 1534-6277
    ISSN 1527-2729
    DOI 10.1007/s11864-021-00922-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Liver and Gastrointestinal Involvement.

    Rosenzweig, Michael / Comenzo, Raymond L

    Hematology/oncology clinics of North America

    2020  Volume 34, Issue 6, Page(s) 1081–1090

    Abstract: Early diagnosis of AL amyloidosis and appreciation of the nutritional and coagulation abnormalities associated with liver and gastrointestinal involvement are critically important in the treatment and management. In cases of severe malabsorption total ... ...

    Abstract Early diagnosis of AL amyloidosis and appreciation of the nutritional and coagulation abnormalities associated with liver and gastrointestinal involvement are critically important in the treatment and management. In cases of severe malabsorption total parenteral nutrition can be extremely helpful as a bridge to organ improvement. Rarely the use of antifibrinolytic agents such as oral aminocaproic acid with transfusion support may control severe bleeding in patients with coagulation abnormalities. It is important to keep in mind that organ improvement should follow in lag phase after the reduction in the pathologic free light chain with treatment. Closely following light chain levels may permit brief holidays from treatment and enable periods of recovery before resuming therapy in patients with prompt early and deep hematologic responses.
    MeSH term(s) Adult ; Female ; Gastrointestinal Diseases/diagnosis ; Gastrointestinal Diseases/etiology ; Gastrointestinal Diseases/metabolism ; Gastrointestinal Diseases/therapy ; Humans ; Immunoglobulin Light Chains/metabolism ; Immunoglobulin Light-chain Amyloidosis/complications ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/metabolism ; Immunoglobulin Light-chain Amyloidosis/therapy ; Liver Diseases/diagnosis ; Liver Diseases/etiology ; Liver Diseases/metabolism ; Liver Diseases/therapy ; Male ; Middle Aged
    Chemical Substances Immunoglobulin Light Chains
    Language English
    Publishing date 2020-10-01
    Publishing country United States
    Document type Case Reports ; Journal Article ; Review
    ZDB-ID 93115-9
    ISSN 1558-1977 ; 0889-8588
    ISSN (online) 1558-1977
    ISSN 0889-8588
    DOI 10.1016/j.hoc.2020.09.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Stigmata of amyloidosis; external manifestations of internal disease.

    Chaulagain, Chakra P / Comenzo, Raymond L

    British journal of haematology

    2019  Volume 186, Issue 1, Page(s) 10

    MeSH term(s) Amyloidosis/diagnosis ; Amyloidosis/pathology ; Christianity ; Humans ; Male
    Language English
    Publishing date 2019-03-27
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 80077-6
    ISSN 1365-2141 ; 0007-1048
    ISSN (online) 1365-2141
    ISSN 0007-1048
    DOI 10.1111/bjh.15885
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  8. Article ; Online: Laboratory-Based Rationale for Targeting the Protein Homeostasis Network in AL Amyloidosis

    Giada Bianchi / Maria Moscvin / Raymond L. Comenzo

    Hemato, Vol 3, Iss 22, Pp 298-

    2022  Volume 317

    Abstract: AL amyloidosis is an incurable plasma cell dyscrasia with limited therapeutic options. The pathogenetic mechanism in AL amyloidosis is the deposition of insoluble fibrillary aggregates of misfolded immunoglobulin (Ig) free light chains (FLC) and ... ...

    Abstract AL amyloidosis is an incurable plasma cell dyscrasia with limited therapeutic options. The pathogenetic mechanism in AL amyloidosis is the deposition of insoluble fibrillary aggregates of misfolded immunoglobulin (Ig) free light chains (FLC) and chaperone proteins in target organs. Therefore, AL amyloidosis is the prototypic, protein-toxicity hematologic disorder. Based on laboratory evidence of increased, constitutive proteotoxic stress, PCs are intrinsically vulnerable to agents that target proteins whose function is to guarantee that nascent polypeptides either reach a functional conformation or are disposed of (proteostasis network). The clinical efficacy of proteasome inhibitors (PIs), such as bortezomib, in the treatment of plasma cell (PC) disorders has provided proof of concept that disrupting protein homeostasis is an effective and generally safe therapeutic approach. Therefore, the intrinsic biology of PC offers us the opportunity to rationally develop therapies that target this distinct proteostasis vulnerability of PC dyscrasias. In this manuscript, we will review the laboratory rationale for the effectiveness of FDA-approved and investigational agents targeting protein homeostasis in AL amyloidosis and related PC disorders.
    Keywords AL amyloidosis ; proteostasis network ; ubiquitin–proteasome system ; therapeutic targets ; proteotoxicity ; protein degradation ; Medicine ; R
    Language English
    Publishing date 2022-04-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article ; Online: AL Amyloidosis: Current Chemotherapy and Immune Therapy Treatment Strategies:

    Bianchi, Giada / Zhang, Yifei / Comenzo, Raymond L

    JACC. CardioOncology

    2021  Volume 3, Issue 4, Page(s) 467–487

    Abstract: Immunoglobulin light chain (AL) amyloidosis is an incurable plasma cell disorder characterized by deposition of fibrils of misfolded immunoglobulin free light chains (FLC) in target organs, leading to failure. Cardiac involvement is common in AL ... ...

    Abstract Immunoglobulin light chain (AL) amyloidosis is an incurable plasma cell disorder characterized by deposition of fibrils of misfolded immunoglobulin free light chains (FLC) in target organs, leading to failure. Cardiac involvement is common in AL amyloidosis and represents the single most adverse prognostic feature. A high index of clinical suspicion with rapid tissue diagnosis and commencement of combinatorial, highly effective cytoreductive therapy is crucial to arrest the process of amyloid deposition and preserve organ function. The clinical use of molecularly targeted drugs, such as proteasome inhibitors and immunomodulatory agents, monoclonal antibodies such as daratumumab, and risk-adjusted autologous stem cell transplant in eligible patients, has radically changed the natural history of AL amyloidosis. Here, we review the state-of-the-art treatment landscape in AL amyloidosis with an eye toward future therapeutic venues to impact the outcome of this devastating illness.
    Language English
    Publishing date 2021-10-19
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 2666-0873
    ISSN (online) 2666-0873
    DOI 10.1016/j.jaccao.2021.09.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Out, Out--Making Amyloid's Candle Briefer.

    Comenzo, Raymond L

    The New England journal of medicine

    2015  Volume 373, Issue 12, Page(s) 1167–1169

    MeSH term(s) Amyloidosis/drug therapy ; Antibodies, Monoclonal/administration & dosage ; Carboxylic Acids/administration & dosage ; Humans ; Pyrrolidines/administration & dosage ; Serum Amyloid P-Component/antagonists & inhibitors
    Chemical Substances Antibodies, Monoclonal ; Carboxylic Acids ; Pyrrolidines ; Serum Amyloid P-Component
    Language English
    Publishing date 2015-09-17
    Publishing country United States
    Document type Comment ; Editorial
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMe1508746
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