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Nodular Pulmonary Amyloidosis with Interstitial Lung Disease—Case Report and Literature Review

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Open Journal of Thoracic Surgery 2024年第2期14卷 40-45页

作者： Efosa Imuetinyan Irshad Soomro Muhammad Hawari Adnan Raza Department of Thoracic Surgery Nottingham University Hospital Nottingham UK Department of Pathology Nottingham University Hospital Nottingham UK

Amyloidosis is a rare spectrum of disease which involves deposition of misfolded extracellular proteins (amyloids) in various body organs leading to progressive organ dysfunction. Clinical presentation can be variable depending on the organ involved and type of protein. Amyloidosis can be classified based on quantity, type, and location of these proteins. Amyloid light-chain amyloidosis develops in the bone marrow, producing abnormal forms of light-chain proteins, which cannot be broken down. These proteins transform into amyloid fibrils and form amyloid deposits in different organs. Pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis;however, it may present as localised pulmonary disease. Localized pulmonary Amyloidosis can present as nodular, cystic, or tracheobronchial amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. This is a case of a 47-year-old female with background history of interstitial lung disease presenting with progressive shortness of breath. Computed tomography scan revealed bilateral pulmonary nodules. The patient was referred to our thoracic surgery team with the suspicion of bronchogenic malignancy with metastasis. Diagnostic video assisted wedge resection was performed for this patient, and histology confirmed pulmonary amyloidosis of nodular type. Amyloid deposition simulates both inflammatory and neoplastic conditions. Definitive diagnosis requires biopsy confirmation therefore early detection and commencing the patient on appropriate treatment pathway may help in symptomatic relief and better outcome.

关键词： Nodular Amyloidosis Interstitial Lung Disease immunomodulatory drugs

Overcoming drug resistance by targeting protein homeostasis in multiple myeloma

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Cancer Drug Resistance 2021年第4期4卷 1028-1046页

作者： Maria Moscvin Matthew Ho Giada Bianchi Department of Medicine Division of HematologyBrigham and Women’s HospitalBostonMA 02115USA Department of Medicine Mayo ClinicRochesterMN 240010USA

Multiple myeloma(MM)is a plasma cell disorder typically characterized by abundant synthesis of clonal immunoglobulin or free light *** incurable,a deeper understanding of MM pathobiology has fueled major therapeutical advances over the past two decades,significantly improving patient *** inhibitors,immunomodulatory drugs,and monoclonal antibodies are among the most effective anti-MM drugs,targeting not only the cancerous cells,but also the bone marrow ***,de novo resistance has been reported,and acquired resistance is inevitable for most patients over time,leading to relapsed/refractory disease and poor *** protein synthesis coupled with impaired/insufficient proteolytic mechanisms makes MM cells exquisitely sensitive to perturbations in protein homeostasis,offering us the opportunity to target this intrinsic vulnerability for therapeutic *** review highlights the scientific rationale for the clinical use of FDA-approved and investigational agents targeting protein homeostasis in MM.

关键词： Multiple myeloma drug resistance proteasome inhibitors immunomodulatory drugs proteostasis endoplasmic reticulum stress unfolded protein response

CRISPR-Cas9 genome-wide screen to unravel mechanisms underlying cancer resistance to targeted therapy

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CRISPR-Cas9 genome-wide screen to unravel mechanisms underly...

First line vs delayed transplantation in myeloma:Certainties and controversies

中国生物化学与分子生物学会2016年全国学术会议

作者： Jiye Liu Shaobing Gao Wenrong Zhou Min Long Chenlu Geng Zhengang Peng Xi Li Yong Cang Life Sciences Institute Zhejiang University Oncology Business Unit Wu Xi App Tec Co. Ltd.

Multiple myeloma(MM)is a malignant plasma cell neoplasia,accounting for about 10%hematopoietic cancers and 1%of all *** of MM patients has significantly improved after the approved treatment with the proteasome inhibitor bortezomib and immunomodulatory drugs(IMi Ds)including thalidomide and its derivatives lenalidomide and *** Ds were recently shown to bind and

关键词： CRISPR screen Cereblon immunomodulatory drugs multiple myeloma

来源： cnki会议评论

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World Journal of Transplantation 2016年第2期6卷 321-330页

作者： Annamaria Brioli Klinik für Innere MedizinⅡ(Abteilung Hämatologie und internistische Onkologie) Universitätsklinikum Jena07740 JenaGermany Istituto di Ematologia Seràgnoli Università degli Studi di Bologna

Since the middle of 1990 s autologous stem cell trans-plantation has been the cornerstone for the treatment of young patients with multiple myeloma(MM). In the last decade the introduction of novel agents such as immunomodulatory drugs(IMi Ds) and proteasome inhibitors(PI), has dramatically changed the therapeutic scenario of this yet incurable disease. Due to the impressive results achieved with IMi Ds and PI both in terms of response rates and in terms of progression free and overall survival, and to the toxicity linked to high dose therapy and autologous stem cell transplantation(ASCT), a burning question nowadays is whether all young patients should be offered autotransplanta-tion up front or if this should be reserved for the time of relapse. This article provides a review of the data available regarding ASCT in MM and of the current opinion of the scientific community regarding its optimal timing.

关键词： Autologous stem cell transplantation immunomodulatory drugs Proteasome inhibitors High dose therapy Multiple myeloma