Review
Biochemistry & Molecular Biology
Yukihiro Saito, Kazufumi Nakamura, Hiroshi Ito
Summary: Cardiac involvement significantly affects the prognosis of patients with systemic amyloidosis. Therapeutic methods have been developed for ATTR amyloidosis and AL amyloidosis, which show cardiac involvement, leading to improved prognosis. However, there is currently no established method for removing deposited amyloid, and further research is needed for protecting cardiovascular cells.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, General & Internal
Emilia Czyzewska, Agnieszka Wisniewska, Anna Waszczuk-Gajda, Olga Ciepiela
Summary: The study indicates that the presence of monoclonal lambda chains in patients with AL amyloidosis may be associated with more severe damage to cardiomyocytes and dysfunction of the myocardium.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Nabil Belfeki, Nouha Ghriss, Mehran Monchi, Cyrus Moini
Summary: Cardiac amyloidosis is characterized by the accumulation of misfolded proteins in the heart. Transthyretin and light chain amyloidosis are the most common causes. Recent studies have shown an increasing incidence of this condition due to population aging and advancements in diagnostic tools.
Review
Immunology
Gemma Martinez-Rivas, Sebastien Bender, Christophe Sirac
Summary: Monoclonal immunoglobulin light chain amyloidosis is a rare but severe disease characterized by the deposition of amyloid fibrils in organs. The mechanisms and pathophysiology of this disease are still unclear. The development of reliable animal models for studying AL amyloidosis has proven to be challenging, hampering research efforts.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Cell Biology
Giovanni Palladini, Paolo Milani, Fabio Malavasi, Giampaolo Merlini
Summary: AL amyloidosis is caused by a small B cell clone producing toxic light chains, and the addition of daratumumab to standard treatment has shown promising results in increasing hematologic response rates and organ responses. Anti-CD38 antibodies like daratumumab offer new powerful tools for treating AL amyloidosis, potentially becoming new standards of care.
Review
Oncology
Linchun Xu, Yongzhong Su
Summary: Immunoglobulin light chain amyloidosis (AL) is a chronic plasma cell disorder characterized by FLC misfolding and amyloid deposition, classified into non-hyperdiploidy and hyperdiploidy subtypes with distinct treatment responses and prognostic outcomes based on cytogenetic patterns. Other genetic factors, such as specific immunoglobulin light chain germline genes and somatic mutations, also contribute to amyloid formation in AL.
EXPERIMENTAL HEMATOLOGY & ONCOLOGY
(2021)
Editorial Material
Gastroenterology & Hepatology
Molly Delk Plummer, Fredric Regenstein
Summary: Light chain deposition disease is a rare condition often associated with renal dysfunction, but can also affect the liver. There are few clinical case reports of liver involvement in LCDD.
Article
Medicine, General & Internal
Mi-Hyang Jung, Suyon Chang, Eun Ji Han, Jong-Chan Youn
Summary: This article summarizes the role of multimodal imaging and biomarkers in the diagnosis, risk stratification, and treatment monitoring of cardiac amyloidosis.
Article
Ecology
Kayleigh C. Hauri, Andrea E. Glassmire, William C. Wetzel
Summary: Cultivar mixtures have been studied as a means to control pests, with plant chemical diversity influencing interactions between herbivores and predators, and different classes of chemical diversity having varied effects on pest suppression.
ECOLOGICAL APPLICATIONS
(2021)
Article
Immunology
Jipeng Yan, Jin Zhao, Xiaoxuan Ning, Yunlong Qin, Yan Xing, Yuwei Wang, Qing Jia, Boyong Huang, Rui Ma, Changhui Lei, Meilan Zhou, Zixian Yu, Yumeng Zhang, Wei-Feng Guo, Shiren Sun
Summary: This study demonstrates for the first time the alterations of gut microbiota in patients with AL amyloidosis and successfully establishes and validates a microbial-based diagnostic model. This provides a foundation for future research on microbe-based strategies for diagnosis and treatment in patients with AL amyloidosis.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Brett W. Sperry, Julie A. Khoury, Shahzad Raza, Julie L. Rosenthal
Summary: Amyloidosis is a multisystem disease that requires coordinated care and treatment involving multiple specialists. Establishing comprehensive amyloidosis programs within healthcare institutions can help improve patient care through multidisciplinary collaboration, clear program goals, institutional support, and focus on program growth and development. The creation and growth of such programs have the potential to increase awareness of the disease and benefit both patients and institutions.
HEART FAILURE REVIEWS
(2022)
Article
Immunology
Ziqi Long, Bethan Phillips, Daniel Radtke, Michael Meyer-Hermann, Oliver Bannard
Summary: Research has found that cyclic reentry initiation and B cell refueling are independently regulated in germinal centers (GCs). Cyclic reentry initiation is associated with B cell receptor affinity, while B cell refueling is limited by both T cell affinity and cells' intrinsic antigen-acquiring abilities. This less binary selection mechanism supports complex antibody maturation pathways and clonal diversity in GCs.
SCIENCE IMMUNOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Karl P. Phillips, Joanne Cable, Ryan S. Mohammed, Sebastian Chmielewski, Karolina J. Przesmycka, Cock van Oosterhout, Jacek Radwan
Summary: This study investigated the roles of local adaptation and host genetic composition in explaining differences in susceptibility to infection using controlled infection experiments with wild-caught guppies and their ectoparasite Gyrodactylus turnbulli. The study found a significant negative relationship between individual-level functional MHC variability and infection, suggesting that population-level differences in host infection susceptibility probably reflect variation in parasite selective pressure and/or host evolutionary potential. The research also showed that greater values of host population genetic variability metrics broadly aligned with lower population mean infection intensity, with the best alignments associated with major histocompatibility complex (MHC) supertypes.
Article
Hematology
Charlie N. Saunders, Subhayan Chattopadhyay, Stefanie Huhn, Niels Weinhold, Per Hoffmann, Markus M. Noethen, Karl-Heinz Joeckel, Boerge Schmidt, Stefano Landi, Hartmut Goldschmidt, Paolo Milani, Giampaolo Merlini, Dorota Rowcieno, Philip Hawkins, Ute Hegenbart, Giovanni Palladini, Ashutosh Wechalekar, Stefan O. Schoenland, Asta Foersti, Richard Houlston, Kari Hemminki
Summary: In this study, Mendelian randomization analysis identified associations between genetically predicted increased monocyte counts and the tumor necrosis factor receptor superfamily member 17 gene with AL amyloidosis risk. Additionally, potential associations with TNFRSF members 6 and 19L were also observed. The findings suggest that high circulating levels of monocytes and TNFRSF proteins may be risk factors for AL amyloidosis, providing insight into the disease's etiology.
Article
Gastroenterology & Hepatology
Taotao Zhou, Henrike Lenzen, Leona Dold, Bennet Bundgens, Heiner Wedemeyer, Michael P. Manns, Maria A. Gonzalez-Carmona, Christian P. Strassburg, Tobias J. Weismuller
Summary: Patients with PSC and elevated sIgG4 should be considered as a distinct subgroup, characterized by different clinical and cholangiographical features and are associated with an inferior outcome.
LIVER INTERNATIONAL
(2021)
Article
Hematology
Aldo A. Acosta-Medina, Ann M. Moyer, Ronald S. Go, Maria Alice V. Willrich, Fernando C. Fervenza, Nelson Leung, Christianne Bourlon, Jeffrey L. Winters, Grant M. Spears, Sandra C. Bryant, Meera Sridharan
Summary: Eculizumab is effective for complement-mediated thrombotic microangiopathy. Discontinuation of therapy does not universally lead to relapse. This study aimed to assess the role of complement genetic variants in predicting recurrence after Eculizumab withdrawal. A total of 280 patients were included, with CFH and MCP/CD46 being the most commonly identified genetic variants. The relapse rate after discontinuation was 29.6%, with CFH and MCP/CD46 variants in canonical splice regions having the highest relapse rates.
Article
Hematology
Matthew Ho, Saurabh Zanwar, Francis K. Buadi, Sikander Ailawadhi, Jeremy Larsen, Leif Bergsagel, Moritz Binder, Asher Chanan-Khan, David Dingli, Angela Dispenzieri, Rafael Fonseca, Morie A. Gertz, Wilson Gonsalves, Ronald S. Go, Suzanne Hayman, Prashant Kapoor, Taxiarchis Kourelis, Martha Q. Lacy, Nelson Leung, Yi Lin, Eli Muchtar, Vivek Roy, Taimur Sher, Rahma Warsame, Amie Fonder, Miriam Hobbs, Yi L. Hwa, Robert A. Kyle, S. Vincent Rajkumar, Shaji Kumar
Summary: Patients with multiple myeloma have a lower efficacy from COVID-19 vaccination and a high rate of mortality from COVID-19 in hospitalized patients. The study found a relatively low infection rate but a quarter of the COVID-19 infections were severe. Treatment with CD38 antibody, cardiac and pulmonary comorbidities were independent predictors for ICU admission. Cardiac comorbidity was an independent predictor of mortality, while MM/AL in remission was associated with lower mortality.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Oncology
Marco Dicanio, Matteo Giaccherini, Alyssa Clay-Gilmour, Angelica Macauda, Juan Sainz, Mitchell J. Machiela, Malwina Rybicka-Ramos, Aaron D. Norman, Agata Tyczynska, Stephen J. Chanock, Torben Barington, Shaji K. Kumar, Parveen Bhatti, Wendy Cozen, Elizabeth E. Brown, Anna Suska, Eva K. Haastrup, Robert Z. Orlowski, Marek Dudzinski, Ramon Garcia-Sanz, Marcin Kruszewski, Joaquin Martinez-Lopez, Katia Beider, Elzbieta Iskierka-Jazdzewska, Matteo Pelosini, Sonja Berndt, Malgorzata Razny, Krzysztof Jamroziak, S. Vincent Rajkumar, Artur Jurczyszyn, Annette Juul Vangsted, Pilar Garrido Collado, Ulla Vogel, Jonathan N. Hofmann, Mario Petrini, Aleksandra Butrym, Susan L. Slager, Elad Ziv, Edyta Subocz, Graham G. Giles, Niels Frost Andersen, Grzegorz Mazur, Marzena Watek, Fabienne Lesueur, Michelle A. T. Hildebrandt, Daria Zawirska, Lene Hyldahl Ebbesen, Herlander Marques, Federica Gemignani, Charles Dumontet, Judit Varkonyi, Gabriele Buda, Arnon Nagler, Agnieszka Druzd-Sitek, Xifeng Wu, Katalin Kadar, Nicola J. Camp, Norbert Grzasko, Rosalie G. Waller, Celine Vachon, Federico Canzian, Daniele Campa
Summary: The aim of this study was to identify novel pleiotropic variants involved in multiple myeloma (MM) risk. Through analysis of 28,684 single nucleotide polymorphisms (SNPs), DNAJB4-rs34517439-A was found to be associated with an increased risk of developing MM.
INTERNATIONAL JOURNAL OF CANCER
(2023)
Article
Hematology
Karan L. Chohan, Jonas Paludo, Nishanth Vallumsetla, Dirk Larson, Rebecca L. King, Rong He, Wilson Gonsalves, David Inwards, Thomas E. Witzig, Abhisek Swaika, Tania Jain, Nelson Leung, Sikander Ailawadhi, Craig B. Reeder, Martha Q. Lacy, S. Vincent Rajkumar, Shaji Kumar, Robert A. Kyle, Morie A. Gertz, Stephen M. Ansell, Prashant Kapoor
Summary: Waldenstrom macroglobulinemia (WM) is a rare, indolent lymphoma that mostly affects the elderly. A study compared young WM patients to their older counterparts and found that young patients have lower overall survival and higher death attributable to WM. The treatment strategies for WM have not improved the outcome of young patients, and the impact of Bruton tyrosine kinase inhibitors on this patient population is still unknown.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Nadine Abdallah, Angela Dispenzieri, Eli Muchtar, Francis K. K. Buadi, Prashant Kapoor, Martha Q. Q. Lacy, Yi L. L. Hwa, Amie Fonder, Miriam A. A. Hobbs, Suzanne R. R. Hayman, Nelson Leung, David Dingli, Ronald S. S. Go, Yi Lin, Wilson I. I. Gonsalves, Moritz Binder, Taxiarchis Kourelis, Rahma Warsame, Robert A. A. Kyle, S. Vincent Rajkumar, Morie A. A. Gertz, Shaji K. K. Kumar
Summary: After long-term follow-up, there is no clear evidence to support the benefit of doxycycline in the post-transplant setting.
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
(2023)
Article
Oncology
Bharat Nandakumar, Francis Baffour, Nadine H. H. Abdallah, Shaji K. K. Kumar, Angela Dispenzieri, Francis K. K. Buadi, David Dingli, Martha Q. Q. Lacy, Suzanne R. R. Hayman, Prashant Kapoor, Nelson Leung, Amie Fonder, Miriam Hobbs, Yi Lisa Hwa, Eli Muchtar, Rahma Warsame, Taxiarchis V. V. Kourelis, Ronald S. S. Go, Robert A. A. Kyle, Morie A. A. Gertz, S. Vincent Rajkumar, Jason Klug, Panagiotis Korfiatis, Wilson I. I. Gonsalves
Summary: Using a deep learning-based segmentation approach, sarcopenia can be accurately detected and measured in clinical computed tomography (CT) images of patients with newly diagnosed multiple myeloma (NDMM). Sarcopenia significantly affects overall survival in NDMM patients and is an independent adverse prognostic factor.
Editorial Material
Oncology
Jean-Sebastien Claveau, David L. Murray, Angela Dispenzieri, Prashant Kapoor, Moritz Binder, Francis Buadi, David Dingli, Amie Fonder, Morie Gertz, Wilson Gonsalves, Suzanne Hayman, Miriam Hobbs, Yi Lisa Hwa, Taxiarchis Kourelis, Martha Lacy, Nelson Leung, Yi Lin, Rahma Warsame, Robert A. Kyle, Vincent Rajkumar, Shaji K. Kumar
Article
Urology & Nephrology
Naba Farooqui, Mark Zaidi, Lisa Vaughan, Trevor D. McKee, Eram Ahsan, Kevin D. Pavelko, Jose C. Villasboas, Svetomir Markovic, Timucin Taner, Nelson Leung, Haidong Dong, Mariam P. Alexander, Sandra M. Herrmann
Summary: This study found that immune checkpoint inhibitors (ICIs) therapy may lead to acute kidney injury (AKI) and there are no biomarkers currently available to distinguish AKI caused by ICIs from other causes. The urine levels of cytokines such as TNF-a, IL-2, and IL-10 were significantly higher in AKI-ICI patients. Therefore, these T-cell responses and their respective cytokines may be helpful in differentiating AKI-ICI from AKI-other.
KIDNEY INTERNATIONAL REPORTS
(2023)
Letter
Hematology
Nate Gipe, Nelson Leung, Terra Lasho, Abhishek Mangaonkar, Hassan Alkhateeb, Aref Al-Kali, Naseema Gangat, William Hogan, Kristen McCullough, Ayalew Tefferi, Mariam Priya Alexander, Mrinal M. Patnaik
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Oncology
Nelson Leung, S. Vincent Rajkumar
Summary: Light chain cast nephropathy (LCCN) is a significant cause of acute kidney injury (AKI) in patients with multiple myeloma (MM) and is now considered as a defining event of myeloma. While long-term prognosis has improved, short-term mortality remains higher in patients with LCCN if renal failure is not reversed. Rapid reduction of involved serum free light chain is critical for renal function recovery. Treatment algorithm for MM patients with biopsy-proven LCCN or ruling out other causes of AKI is provided based on randomized trial data whenever available. Clinical trial enrollment is recommended prior to following the treatment algorithm.
BLOOD CANCER JOURNAL
(2023)
Letter
Oncology
Rahul Banerjee, Bo Wang, Larry D. Anderson, Georgia Mccaughan, Nikita Mehra, Andrew J. Cowan, S. Vincent Rajkumar, Gurbakhash Kaur
BLOOD CANCER JOURNAL
(2023)
Editorial Material
Urology & Nephrology
Sheron Latcha, Nelson Leung
Review
Immunology
Hans-Joachim Anders, A. Richard Kitching, Nelson Leung, Paola Romagnani
Summary: 'Glomerulonephritis' (GN) refers to a group of immune-mediated disorders characterized by inflammation of the kidney's filtration units. Current classification is based on histopathological lesion patterns, but these patterns do not align well with the diverse pathological mechanisms and do not guide optimal therapy. Instead, a proposed immunopathogenesis-based classification groups GN disorders into five categories, allowing for more targeted and effective treatments.
NATURE REVIEWS IMMUNOLOGY
(2023)
Article
Urology & Nephrology
Jason N. Barreto, Erin F. Barreto, Kristin C. Mara, Andrew D. Rule, John C. Lieske, Callen D. Giesen, Carrie A. Thompson, Nelson Leung, Thomas E. Witzig, Kianoush B. Kashani