LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 103

Search options

  1. Article ; Online: Case 16-2022: A Man with Fevers, Night Sweats, and a Mediastinal Mass. Reply.

    Fajgenbaum, David C

    The New England journal of medicine

    2022  Volume 387, Issue 8, Page(s) 763

    MeSH term(s) Fever/etiology ; Humans ; Hyperhidrosis ; Male ; Middle Aged ; Sweat ; Sweating
    Language English
    Publishing date 2022-07-21
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMc2208384
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  2. Article: How we manage idiopathic multicentric Castleman disease.

    Brandstadter, Joshua D / Fajgenbaum, David C

    Clinical advances in hematology & oncology : H&O

    2022  Volume 20, Issue 9, Page(s) 564–571

    MeSH term(s) Castleman Disease/diagnosis ; Castleman Disease/drug therapy ; Humans
    Language English
    Publishing date 2022-09-20
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2271951-9
    ISSN 1543-0790
    ISSN 1543-0790
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  3. Article ; Online: Cytokine Storm. Reply.

    Fajgenbaum, David C / June, Carl H

    The New England journal of medicine

    2021  Volume 384, Issue 16, Page(s) e59

    MeSH term(s) COVID-19 ; Cytokine Release Syndrome ; Cytokines ; Humans
    Chemical Substances Cytokines
    Language English
    Publishing date 2021-04-07
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMc2036236
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  4. Article ; Online: Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.

    Fajgenbaum, David C

    Hematology. American Society of Hematology. Education Program

    2018  Volume 2018, Issue 1, Page(s) 318–325

    Abstract: Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy ( ... ...

    Abstract Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy (MCD) with systemic inflammation, cytopenias, and life-threatening multiple organ dysfunction resulting from a cytokine storm often driven by interleukin 6 (IL-6). Uncontrolled human herpesvirus-8 (HHV-8) infection causes approximately 50% of MCD cases, whereas the etiology is unknown in the remaining HHV-8-negative/idiopathic MCD cases (iMCD). The limited understanding of etiology, cell types, and signaling pathways involved in iMCD has slowed development of treatments and contributed to historically poor patient outcomes. Here, recent progress for diagnosing iMCD, characterizing etio-pathogenesis, and advancing treatments are reviewed. Several clinicopathological analyses provided the evidence base for the first-ever diagnostic criteria and revealed distinct clinical subtypes: thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS), which are both observed all over the world. In 2014, the anti-IL-6 therapy siltuximab became the first iMCD treatment approved by the US Food and Drug Administration, on the basis of a 34% durable response rate; consensus guidelines recommend it as front-line therapy. Recent cytokine and proteomic profiling has revealed normal IL-6 levels in many patients with iMCD and potential alternative driver cytokines. Candidate novel genomic alterations, dysregulated cell types, and signaling pathways have also been identified as candidate therapeutic targets. RNA sequencing for viral transcripts did not reveal novel viruses, HHV-8, or other viruses pathologically associated with iMCD. Despite progress, iMCD remains poorly understood. Further efforts to elucidate etiology, pathogenesis, and treatment approaches, particularly for siltuximab-refractory patients, are needed.
    MeSH term(s) Antibodies, Monoclonal/therapeutic use ; Castleman Disease/diagnosis ; Castleman Disease/drug therapy ; Castleman Disease/etiology ; Castleman Disease/metabolism ; Herpesviridae Infections/complications ; Herpesviridae Infections/diagnosis ; Herpesviridae Infections/drug therapy ; Herpesviridae Infections/metabolism ; Herpesvirus 8, Human/metabolism ; Humans ; Interleukin-6/antagonists & inhibitors ; Interleukin-6/metabolism ; Practice Guidelines as Topic ; Signal Transduction/drug effects
    Chemical Substances Antibodies, Monoclonal ; IL6 protein, human ; Interleukin-6 ; siltuximab (T4H8FMA7IM)
    Language English
    Publishing date 2018-11-30
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ZDB-ID 2084287-9
    ISSN 1520-4383 ; 1520-4391
    ISSN (online) 1520-4383
    ISSN 1520-4391
    DOI 10.1182/asheducation-2018.1.318
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  5. Article ; Online: Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.

    Fajgenbaum, David C

    Blood

    2018  Volume 132, Issue 22, Page(s) 2323–2330

    Abstract: Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy ( ... ...

    Abstract Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy (MCD) with systemic inflammation, cytopenias, and life-threatening multiple organ dysfunction resulting from a cytokine storm often driven by interleukin 6 (IL-6). Uncontrolled human herpesvirus-8 (HHV-8) infection causes approximately 50% of MCD cases, whereas the etiology is unknown in the remaining HHV-8-negative/idiopathic MCD cases (iMCD). The limited understanding of etiology, cell types, and signaling pathways involved in iMCD has slowed development of treatments and contributed to historically poor patient outcomes. Here, recent progress for diagnosing iMCD, characterizing etio-pathogenesis, and advancing treatments are reviewed. Several clinicopathological analyses provided the evidence base for the first-ever diagnostic criteria and revealed distinct clinical subtypes: thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS), which are both observed all over the world. In 2014, the anti-IL-6 therapy siltuximab became the first iMCD treatment approved by the US Food and Drug Administration, on the basis of a 34% durable response rate; consensus guidelines recommend it as front-line therapy. Recent cytokine and proteomic profiling has revealed normal IL-6 levels in many patients with iMCD and potential alternative driver cytokines. Candidate novel genomic alterations, dysregulated cell types, and signaling pathways have also been identified as candidate therapeutic targets. RNA sequencing for viral transcripts did not reveal novel viruses, HHV-8, or other viruses pathologically associated with iMCD. Despite progress, iMCD remains poorly understood. Further efforts to elucidate etiology, pathogenesis, and treatment approaches, particularly for siltuximab-refractory patients, are needed.
    MeSH term(s) Adrenal Cortex Hormones/therapeutic use ; Animals ; Antibodies, Monoclonal/therapeutic use ; Castleman Disease/diagnosis ; Castleman Disease/drug therapy ; Castleman Disease/etiology ; Castleman Disease/immunology ; Disease Management ; Herpesviridae Infections/complications ; Herpesvirus 8, Human/isolation & purification ; Humans ; Immunologic Factors/therapeutic use ; Interleukin-6/antagonists & inhibitors ; Interleukin-6/immunology ; Rituximab/therapeutic use
    Chemical Substances Adrenal Cortex Hormones ; Antibodies, Monoclonal ; Immunologic Factors ; Interleukin-6 ; Rituximab (4F4X42SYQ6) ; siltuximab (T4H8FMA7IM)
    Language English
    Publishing date 2018-11-28
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood-2018-05-848671
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  6. Article ; Online: A model for crowdsourcing high-impact research questions for Castleman disease and other rare diseases.

    Korsunska, Ania / Repasky, Mileva / Zuccato, Mary / Fajgenbaum, David C

    Orphanet journal of rare diseases

    2023  Volume 18, Issue 1, Page(s) 75

    Abstract: Background: There are approximately 10,000 rare diseases that affect around 30,000,000 individuals in the U.S.A., most of which do not have an FDA-approved treatment. This fact highlights the failure of traditional research approaches to overcome the ... ...

    Abstract Background: There are approximately 10,000 rare diseases that affect around 30,000,000 individuals in the U.S.A., most of which do not have an FDA-approved treatment. This fact highlights the failure of traditional research approaches to overcome the unique challenges of developing rare disease treatments. The Castleman Disease Collaborative Network was founded in 2012 to advance research and treatments for Castleman disease, a rare and deadly disease that involves the immune system attacking the body's vital organs for an unknown cause. It has spearheaded a novel strategy for advancing biomedical research, the Collaborative Network Approach. This approach consists of eight steps, one of which is to identify and prioritize high-impact research questions through crowdsourcing ideas from the entire community of stakeholders: patients, loved ones, physicians, and researchers. Rather than hoping that the right researcher will apply for the right research project at the right time, crowdsourcing high-priority research projects into a research strategy ensures that the most high-impact, patient-centered studies are prioritized. The Castleman Disease Collaborative Network launched an initiative in 2021 to systematically generate this list of community-directed studies to focus Castleman disease research efforts.
    Results: The Castleman Disease Collaborative Network was able to successfully create a patient-centered research agenda through engaging the entire community of stakeholders. The community contributed important questions about Castleman disease, which were prioritized and reviewed by our Scientific Advisory Board, and the result was a finalized list of studies that address these prioritized questions. We were also able to generate a best practices list which can serve as a model that can be utilized for other rare diseases.
    Conclusion: Creating a patient-centered research agenda through crowdsourcing research ideas from the community is one of the most important ways that the Castleman Disease Collaborative Network operationalizes its commitment to keeping patients at the center of research and we hope that by sharing these insights we can assist other rare disease organizations to pursue a patient-centric approach.
    MeSH term(s) Humans ; Castleman Disease ; Rare Diseases ; Crowdsourcing ; Biomedical Research ; Research Design
    Language English
    Publishing date 2023-04-11
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2225857-7
    ISSN 1750-1172 ; 1750-1172
    ISSN (online) 1750-1172
    ISSN 1750-1172
    DOI 10.1186/s13023-023-02678-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  7. Article ; Online: Commentary on A Case of Rapid Deterioration with Marked Hypergammaglobulinemia.

    Fajgenbaum, David C / Phillips, Alexis D

    Clinical chemistry

    2020  Volume 66, Issue 11, Page(s) 1378–1379

    MeSH term(s) Humans ; Hypergammaglobulinemia/complications ; Hypergammaglobulinemia/diagnosis ; Plasma Cells
    Language English
    Publishing date 2020-11-03
    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/hvaa156
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  8. Article ; Online: Cytokine Storm.

    Fajgenbaum, David C / June, Carl H

    The New England journal of medicine

    2020  Volume 383, Issue 23, Page(s) 2255–2273

    MeSH term(s) Autoimmune Diseases/complications ; Bacterial Infections/complications ; Blood Proteins/physiology ; COVID-19/complications ; Chemokines/physiology ; Cytokine Release Syndrome/etiology ; Cytokine Release Syndrome/physiopathology ; Cytokine Release Syndrome/therapy ; Cytokines/immunology ; Cytokines/physiology ; Humans ; Interleukins/physiology ; Virus Diseases/complications
    Chemical Substances Blood Proteins ; Chemokines ; Cytokines ; Interleukins
    Language English
    Publishing date 2020-12-02
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMra2026131
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  9. Article ; Online: Teaching Old Drugs New Tricks: Statins for COVID-19?

    Fajgenbaum, David C / Rader, Daniel J

    Cell metabolism

    2020  Volume 32, Issue 2, Page(s) 145–147

    Abstract: The COVID-19 pandemic has driven unprecedented efforts to identify existing treatments that can be quickly and effectively repurposed to reduce morbidity and mortality. In this issue of Cell Metabolism, Zhang et al. (2020) report an association between ... ...

    Abstract The COVID-19 pandemic has driven unprecedented efforts to identify existing treatments that can be quickly and effectively repurposed to reduce morbidity and mortality. In this issue of Cell Metabolism, Zhang et al. (2020) report an association between statin use and improved outcomes in a large observational study of hospitalized COVID-19 patients. Given the widespread availability, low cost, and safety of statins, this promising result should be further investigated in randomized controlled trials.
    MeSH term(s) Betacoronavirus/drug effects ; COVID-19 ; Coronavirus Infections/drug therapy ; Cytokine Release Syndrome/drug therapy ; Drug Repositioning/methods ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use ; Observational Studies as Topic ; Pandemics ; Pneumonia, Viral/drug therapy ; SARS-CoV-2
    Chemical Substances Hydroxymethylglutaryl-CoA Reductase Inhibitors
    Keywords covid19
    Language English
    Publishing date 2020-07-24
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2176834-1
    ISSN 1932-7420 ; 1550-4131
    ISSN (online) 1932-7420
    ISSN 1550-4131
    DOI 10.1016/j.cmet.2020.07.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  10. Article ; Online: Overview of Castleman disease.

    Dispenzieri, Angela / Fajgenbaum, David C

    Blood

    2020  Volume 135, Issue 16, Page(s) 1353–1364

    Abstract: Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and ... ...

    Abstract Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), human herpes virus-8 (HHV8)-associated MCD (HHV8-MCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD (POEMS-MCD). iMCD can be further subclassified into iMCD-thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS). Advances in diagnosis, classification, pathogenesis, and therapy are substantial since the original description of UCD by Benjamin Castleman in 1954. The advent of effective retroviral therapy and use of rituximab in HHV8-MCD have improved outcomes in HHV8-MCD. Anti-interleukin-6-directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases. Much of the recent progress has been coordinated by the Castleman Disease Collaborative Network (CDCN), and further progress will be made by continued engagement of physicians, scientists, and patients. Progress can also be facilitated by encouraging patients to self-enroll in the CDCN's ACCELERATE natural history registry (#NCT02817997; www.CDCN.org/ACCELERATE).
    MeSH term(s) Animals ; Castleman Disease/diagnosis ; Castleman Disease/pathology ; Castleman Disease/therapy ; Castleman Disease/virology ; Herpesviridae Infections/complications ; Herpesviridae Infections/diagnosis ; Herpesviridae Infections/therapy ; Herpesviridae Infections/virology ; Herpesvirus 8, Human/drug effects ; Herpesvirus 8, Human/isolation & purification ; Humans ; Immunologic Factors/therapeutic use ; Interleukin-6/antagonists & inhibitors ; Rituximab/therapeutic use
    Chemical Substances Immunologic Factors ; Interleukin-6 ; Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2020-02-19
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2019000931
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

To top