Article
Cardiac & Cardiovascular Systems
Sarah A. M. Cuddy, Michael Chetrit, Madeline Jankowski, Milind Desai, Rodney H. Falk, Rory B. Weiner, Allan L. Klein, Dermot Phelan, Martha Grogan
Summary: This document provides a practical guide for the imaging findings of cardiac amyloidosis, highlighting red flags for diagnosis and offering tips for image acquisition and interpretation. It also offers recommendations for standard reporting of an echocardiogram in a patient with cardiac amyloidosis, based on comprehensive literature from multiple societies.
JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY
(2022)
Article
Oncology
Yang Li, Yanze Cao, Mingxin Zheng, Jiaqi Hu, Wei Yan, Xiaoyu Liu, Aijun Liao, Wei Yang, Jian Li, Huihan Wang
Summary: Based on a large Chinese cohort of AL patients with cardiac involvement, nomogram models were identified to predict cardiac response and OS. These models, which consider treatment-related factors, are individualized and dynamic, and have important clinical application value.
FRONTIERS IN ONCOLOGY
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Keying Bi, Ke Wan, Yuanwei Xu, Jie Wang, Weihao Li, Jiajun Guo, Ziqian Xu, Yangjie Li, Qiao Deng, Wei Cheng, Jiayu Sun, Yucheng Chen
Summary: This study evaluated the clinical and prognostic value of pulmonary transit time (PTT) derived from first-pass perfusion MRI in patients with AL amyloidosis. The results showed that PTT can serve as a new imaging predictor of cardiac involvement and prognosis in AL amyloidosis.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Review
Medicine, General & Internal
Agnieszka Stelmach-Goldys, Monika Zaborek-Lyczba, Jakub Lyczba, Bartosz Garus, Marcin Pasiarski, Paulina Mertowska, Paulina Malkowska, Rafal Hrynkiewicz, Paulina Niedzwiedzka-Rystwej, Ewelina Grywalska
Summary: AL amyloidosis is a systemic disease characterized by the deposition of protein fibers formed from light chains produced by neoplastic clone of plasmocytes. Late diagnosis leads to high mortality rate, and the complex clinical picture and slow progression of the disease contribute to the delayed diagnosis. Early diagnosis and correct identification of the type of amyloidosis are crucial for treatment planning and effectiveness.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Hematology
Mehmet Kemal Samur, Marco Roncador, Anil Aktas Samur, Mariateresa Fulciniti, Abdul Hamid Bazarbachi, Raphael Szalat, Masood A. Shammas, Adam S. Sperling, Paul G. Richardson, Florence Magrangeas, Stephane Minvielle, Aurore Perrot, Jill Corre, Philippe Moreau, Anjan Thakurta, Giovanni Parmigiani, Kenneth C. Anderson, Herve Avet-Loiseau, Nikhil C. Munshi
Summary: High-dose melphalan improves progression-free survival in multiple myeloma, but it causes more mutations in surviving myeloma cells at relapse. These newly acquired mutations are associated with DNA damage and double-stranded breaks, and are predominantly found on the transcribed strand. A machine learning model based on this unique pattern can predict patients who will receive high-dose melphalan with high accuracy.
Article
Clinical Neurology
Min Qian, Lan Qin, Kaini Shen, Hongzhi Guan, Haitao Ren, Yanhuan Zhao, Yuzhou Guan, Daobin Zhou, Bin Peng, Jian Li, Lin Chen
Summary: This study retrospectively reviewed the clinical data of 8 AL amyloidosis patients with peripheral neuropathy as the initial presentation, and found that early recognition and chemotherapy are crucial for better outcomes. Nerve biopsy can help to make the diagnosis.
FRONTIERS IN NEUROLOGY
(2021)
Article
Hematology
Hamza Hassan, Karina Verma, Grace Ferri, Dina Brauneis, Karen Quillen, J. Mark Sloan, Vaishali Sanchorawala, Camille Vanessa Edwards
Summary: High-dose melphalan and autologous stem cell transplantation (HDM/SCT) is well tolerated in older patients with AL amyloidosis. Compared to the mHDM group, patients in the HDM group had a higher proportion of very good partial response at 12 months and longer progression-free survival. However, transplant-related non-hematologic toxicities were more common in the HDM group.
TRANSPLANTATION AND CELLULAR THERAPY
(2022)
Article
Hematology
Jae-Cheol Jo, Jin-Seok Kim, Je-Hwan Lee, Jung-Hee Lee, Seong Nam Im, Sang-Min Lee, Sung-Soo Yoon, In-Ho Kim, Seong Hwa Bae, Yoo Jin Lee, Yunsuk Choi, Won-Sik Lee
Summary: The study evaluated the efficacy and toxicity of the BuEAM high-dose regimen for ASCT in patients with T-cell lymphomas, showing promising results with acceptable toxicity levels and some patients achieving complete remission post-ASCT. The BuEAM regimen demonstrated potential effectiveness across various subtypes of T-cell lymphomas.
ANNALS OF HEMATOLOGY
(2021)
Article
Hematology
Fujia Miao, Guisheng Ren, Jinzhou Guo, Liang Zhao, Weiwei Xu, Xianghua Huang
Summary: This study retrospectively investigated the occurrence and influencing factors of ES in 302 AL amyloidosis patients with renal involvement who underwent autologous stem cell transplantation (ASCT). The incidence of ES was 22.2% and was associated with the estimated glomerular filtration rate at baseline and C-reactive protein level on the day of granulocyte engraftment.
TRANSPLANTATION AND CELLULAR THERAPY
(2023)
Article
Medicine, General & Internal
Liang Zhao, Guisheng Ren, Jinzhou Guo, Wencui Chen, Weiwei Xu, Xianghua Huang
Summary: This study evaluates the clinical characteristics and prognostic factors of hepatic systemic light chain (AL) amyloidosis. The results show that the disease is associated with poor survival and rarely has specific manifestations. Both significant increase of NT-proBNP and hyperbilirubinemia indicate a poor prognosis. Vigilance should be raised to the relevant clinical manifestations, and early diagnosis and timely treatment can improve the prognosis.
ANNALS OF MEDICINE
(2022)
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
Mirela Draghici, Andreea Jercan, Sorina Nicoleta Badelita, Ruxandra Maria Irimia, Alexandra Eugenia Bastian, Camelia Dobrea, Monica Popescu, Daniel Coriu
Summary: This case report highlights a rare manifestation of light chain amyloidosis (AL) amyloidosis, presenting as muscle pseudohypertrophy with involvement of the heart, kidneys, nervous system, and skeletal muscles. The patient received three subsequent lines of therapy over 23 months, resulting in slow hematological remission and resolution of organ dysfunction.
Editorial Material
Hematology
Iuliana Vaxman, Caroline Kaufman, Amir Lerman, Morie A. Gertz
Summary: This article discusses coronary microvascular angina caused by cardiac amyloidosis.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Hematology
Joshua N. Gustine, Andrew Staron, Lisa Mendelson, Tracy Joshi, Deepa M. Gopal, Omar K. Siddiqi, Frederick L. Ruberg, Vaishali Sanchorawala
Summary: Patients with advanced cardiac AL amyloidosis have a poor prognosis. Early hematologic and cardiac responses can prolong survival. This study identified factors predictive of treatment outcomes and survival.
Article
Oncology
Sofia Chatzileontiadou, Thomas Zegkos, Christina Frouzaki, Athanasia Apsemidou, Georgios Efthimiadis, Despoina Parcharidou, Maria Papaioannou
Summary: Cardiac amyloidosis is a rare myocardial disorder characterized by fibril deposition of misfolding proteins. Early and accurate diagnosis is crucial for prompt treatment initiation, as the severity of cardiac involvement is strongly associated with morbidity and mortality. Light chain amyloidosis is a life-threatening disease caused by clonal plasma cells producing amyloidogenic light chain fragments.
FRONTIERS IN ONCOLOGY
(2022)
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)
Article
Oncology
Alexander J. Ryu, Shaji Kumar, Angela Dispenzieri, Robert A. Kyle, S. Vincent Rajkumar, Thomas C. Kingsley
Summary: This study used machine learning and electronic health record data to identify potential drug candidates for modifying the progression risk of MGUS. Various medications were found to be significantly associated with the risk of MGUS progression, which could inform future prospective studies.
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
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