Article
Medicine, General & Internal
Nuria M. Zellweger, Jan Huber, Dimitrios A. Tsakiris, Alexandar Tzankov, Caroline E. Gebhard, Martin Siegemund
Summary: This case report describes a 58-year-old man with both SARS-CoV-2 infection and later development of HLH admitted to the ICU. The patient experienced multiple complications during his ICU stay leading to multiorgan failure and death. Despite being diagnosed with HLH, the extremely high ferritin levels of the patient remained not fully understood.
SWISS MEDICAL WEEKLY
(2021)
Review
Medicine, General & Internal
H. Yildiz, E. Van Den Neste, J. P. Defour, E. Danse, J. C. Yombi
Summary: This review updates knowledge on adult HLH pathophysiology, helps clinicians make early diagnosis and treatment, and identifies various causes. HLH is a life-threatening disorder associated with hematological diseases, infections, and autoimmune diseases. The mortality rate is high, but new therapies show promise.
QJM-AN INTERNATIONAL JOURNAL OF MEDICINE
(2022)
Article
Medicine, General & Internal
Wei-I. Lee, Dipti Talaulikar, Matthew C. Cook
Summary: This study emphasizes the importance of comprehensive application of diagnostic criteria to improve accuracy and timelines of the diagnosis of adult onset HLH.
INTERNAL MEDICINE JOURNAL
(2021)
Article
Pharmacology & Pharmacy
Chunjiang Wang, Zhiqiang Fan, Yang He, Weijin Fang, Wei Sun, Zuojun Li
Summary: Lamotrigine has been found to be related to haemophagocytic lymphohistiocytosis (HLH), but the knowledge is mainly based on case reports. This study evaluated the clinical characteristics of lamotrigine-induced HLH and found that the major clinical features include fever, cytopenia, rash, and hyperferritinaemia.
JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS
(2022)
Review
Hematology
Audi Setiadi, Adi Zoref-Lorenz, Christina Y. Lee, Michael B. Jordan, Luke Y. C. Chen
Summary: Haemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome that can occur with cancer or immune-activating therapies for cancer. Familial HLH is characterized by uncontrolled activation of macrophages and cytotoxic T cells, while the pathophysiology of malignancy-associated HLH is not well understood. Distinguishing HLH from cancer-related abnormalities is challenging and emerging diagnostic tools are discussed. Targeted therapy, including cytokine blockade and JAK-STAT inhibition, may improve outcomes. The framework of cytokine storm syndrome can help understand malignancy-associated HLH.
LANCET HAEMATOLOGY
(2022)
Review
Pediatrics
Marie Meeths, Yenan T. Bryceson
Summary: HLH is a life-threatening hyperinflammatory syndrome with genetic predisposition and associations with variants in genes related to metabolism or immunity. Molecular insights have provided fundamental knowledge of the immune system, deepening our understanding of hyperinflammatory diseases and highlighting new treatment strategies.
Review
Cell Biology
Alexander Kikuchi, Kunwar Singh, Eric Gars, Robert S. Ohgami
Summary: Haemophagocytic lymphohistiocytosis is a complex and often underestimated immune dysregulation syndrome that can occur in various clinical scenarios. Timely and accurate diagnosis is crucial for patient survival, requiring a high level of clinical suspicion. The presence of haemophagocytosis in certain clinical situations should prompt consideration of this syndrome and further testing.
Article
Gastroenterology & Hepatology
Deepika Suresh, Ashley Li, Matthew J. Miller, Karn Wijarnpreecha, Vincent L. Chen
Summary: Metabolic hyperferritinaemia is associated with adverse clinical outcomes and common genetic variants related to liver fibrosis in patients with metabolic dysfunction-associated steatotic liver disease (MASLD).
LIVER INTERNATIONAL
(2023)
Article
Hematology
David Kuron, Jakob Christoph Voran, Friedrich Alexander von Samson-Himmelstjerna, Claudia Baldus, Ulrich Kunzendorf, Kevin Schulte, Benedikt Kolbrink
Summary: By retrospectively analyzing German HLH patients, it was found that the incidence and related mortality of HLH have significantly increased, surpassing previous expectations.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Gastroenterology & Hepatology
Edouard Bardou-Jacquet, Houda Hamdi-Roze, Anita Paisant, Marie Decraecker, Marc Bourliere, Nathalie Ganne-Carrie, Victor de Ledinghen, Christophe Bureau
Summary: Increased serum ferritin is a common reason for referral, requiring thorough evaluation to avoid unnecessary exploration and inaccurate diagnosis. Factors and tools relevant to this situation must be known by clinicians. Magnetic resonance imaging is a reference method for accurately determining liver iron content.
CLINICS AND RESEARCH IN HEPATOLOGY AND GASTROENTEROLOGY
(2022)
Review
Pediatrics
Gritta E. Janka, Maurizio Arico
Summary: Familial haemophagocytic lymphohistiocytosis (FHL) is an inherited immune deficiency characterized by defective cytotoxicity of natural killer cells and cytotoxic T lymphocytes. Early diagnosis is crucial due to the rapidly fatal nature of the disease. With current standard-of-care therapy involving etoposide and corticosteroids followed by HSCT, FHL has become a curable disease, and further advancements in cytokine-directed therapy and less toxic conditioning regimens could improve cure rates.
Article
Hematology
Timothy J. Porter, Ana Lazarevic, Jamie E. Ziggas, Ephraim Fuchs, Kiryoung Kim, Helen Byrnes, Leo Luznik, Javier Bolanos-Meade, Syed Abbas Ali, Nirali N. Shah, Nina Wagner-Johnston, Tania Jain
Summary: CAR-HLH, a type of haemophagocytic lymphohistiocytosis-like toxicity that occurs following CAR-T cell therapy, is described in three patients in this report. Prompt recognition and initiation of potentially life-saving treatment for CAR-HLH is crucial.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Editorial Material
Cardiac & Cardiovascular Systems
Defne Guenes Ergi, Ander Dorken Gallastegi, Eren Arslan, Umit Kahraman
Summary: Left ventricular assist devices provide circulatory support for heart failure patients while waiting for a donor heart. Complications such as coagulation disorders and infections are common with prolonged therapy. This case illustrates a rare occurrence of hemophagocytosis in a patient with a left ventricular assist device, which was successfully treated with intravenous immunoglobulin and methylprednisolone, allowing the patient to become eligible for heart transplantation again.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2021)
Article
Oncology
Lin Zhao, Hui Yang, Wei-ying Qu, Ying-jia Lu, Zhou Feng
Summary: Haemophagocytic lymphohistiocytosis (HLH) is a cytokine-driven inflammatory syndrome caused by uncontrolled hypersecretion of inflammatory cytokines. Conventional first-line treatment for HLH included HLH-94 and HLH-2004 regimens. We reported two cases of HLH, one caused by natural killer (NK)/T-cell lymphoma and another associated with missense variants in the perforin 1 gene. They both received the ruxolitinib plus dexamethasone protocol and had a rapid response to treatment without obvious adverse effects. Our report indicates that treatment with ruxolitinib plus dexamethasone might be a potential option for HLH, and clinical trials warrant further investigation. In addition, the detection of HLH-related genes is necessary for the identification of late-onset familial HLH in certain settings.
FRONTIERS IN ONCOLOGY
(2023)
Article
Medicine, General & Internal
Joe West, Tim R. Card, Mark J. Bishton, Peter Lanyon, Lu Ban, Mary Bythell, Lucy Elliss-Brookes, Jessica J. Manson, Vasanta Nanduri, Judith Rankin, Rachel S. Tattersall, Colin J. Crooks
Summary: The study found an increase in the incidence of HLH in England between 2000 and 2016, with patients of all ages affected and significant differences in one-year survival rates.
JOURNAL OF INTERNAL MEDICINE
(2022)
Letter
Medical Laboratory Technology
Grace van der Gugten, Andre Mattman, Gordon Ritchie, Luke Y. C. Chen, Alex Chin, Daniel T. Holmes, John R. Mills, Lokinendi V. Rao
CLINICAL CHEMISTRY
(2021)
Letter
Critical Care Medicine
Luke Y. C. Chen, Ryan L. Hoiland, Sophie Stukas, Cheryl L. Wellington, Mypinder S. Sekhon
LANCET RESPIRATORY MEDICINE
(2021)
Editorial Material
Medicine, General & Internal
Kevin Afra, Luke Y. C. Chen, David Sweet
CANADIAN MEDICAL ASSOCIATION JOURNAL
(2021)
Editorial Material
Hematology
Ryan J. Stubbins, Eric McGinnis, Bhupinder Johal, Luke Y. C. Chen, Lorena Wilson, Daniela Ospina Cardona, Thomas J. Neyill
Review
Hematology
Audi Setiadi, Adi Zoref-Lorenz, Christina Y. Lee, Michael B. Jordan, Luke Y. C. Chen
Summary: Haemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome that can occur with cancer or immune-activating therapies for cancer. Familial HLH is characterized by uncontrolled activation of macrophages and cytotoxic T cells, while the pathophysiology of malignancy-associated HLH is not well understood. Distinguishing HLH from cancer-related abnormalities is challenging and emerging diagnostic tools are discussed. Targeted therapy, including cytokine blockade and JAK-STAT inhibition, may improve outcomes. The framework of cytokine storm syndrome can help understand malignancy-associated HLH.
LANCET HAEMATOLOGY
(2022)
Editorial Material
Medicine, General & Internal
Ryan J. Stubbins, Hannah Cherniawsky, Luke Y. C. Chen, Thomas J. Nevill
CANADIAN MEDICAL ASSOCIATION JOURNAL
(2022)
Article
Urology & Nephrology
Aria Jazdarehee, Azin Ahrari, Drew Bowie, Silvia D. Chang, Henry Tran, Shahin Jamal, Luke Y. C. Chen, Karen C. Tran
Summary: This is a rare case of IgG4-related prostatitis. The resolution of symptoms with steroid treatment highlights the importance of considering this condition in young individuals.
Article
Hematology
Agnes Y. Y. Lee, Muntadhar Al Moosawi, Erica A. Peterson, Rita K. McCracken, Steven K. W. Wong, Hamish Nicolson, Vicky Chan, Tyler Smith, Michelle P. Wong, Lauren J. Lee, Cameron Griffiths, Bhavdeep Rahal, Stephen Parkin, Kevin Afra, Kimberley Ambler, Luke Y. C. Chen, Thalia S. Field, Heather C. Lindsay, Martin Lavoie, Charles Li, David Migneault, Monika Naus, Jolanta Piszczek, Poupak Rahmani, Gayatri Sreenivasan, Tony Wan, Adrian Yee, Leslie Zypchen, David Sweet
Summary: Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare complication after adenoviral vector vaccination for SARS-CoV-2. A clinical care pathway was developed in British Columbia, Canada, to guide clinicians in evaluating patients with thrombocytopenia or thrombosis symptoms for VITT, including PF4-ELISA testing for diagnosis.
Review
Rheumatology
Angelina Marinkovic, Leslie N. Zypchen, Jonathan Chan, Luke Y. C. Chen, Stephen Parkin
Summary: Monoclonal proteins can provide important diagnostic information for non-malignant systemic inflammatory disorders. Certain rare conditions, known as monoclonal gammopathies of clinical significance (MGCS), are associated with variable concentrations of monoclonal proteins, systemic symptoms, and organ dysfunction. This review focuses on distinguishing MGCS from other conditions and highlights four rare MGCS that rheumatologists should be aware of.
LANCET RHEUMATOLOGY
(2022)
Article
Hematology
Amanallah Montazeripouragha, Christine M. Campbell, James Russell, Nadia Medvedev, Daniel R. Owen, Alison Harris, Fergal Donnellan, Iain McCormick, David C. Fajgenbaum, Luke Y. C. Chen
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Review
Cell Biology
Ryan Henrie, Hannah Cherniawsky, Krista Marcon, Eric J. Zhao, Angelina Marinkovic, Persia Pourshahnazari, Stephen Parkin, Luke Y. C. Chen
Summary: This article reviews recent clinical and translational advances in three categories of hematological inflammatory diseases and discusses the important information in initial diagnosis.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
(2022)
Article
Health Care Sciences & Services
Sophie Y. Wang, Paola Cantarelli, Oliver Groene, Tom Stargardt, Nicola Belle
Summary: This study investigated the relative importance of patient expectations, clinical uncertainty, and past behavior on the antibiotic prescribing decisions of hospital-based physicians in Tuscany, Italy. The results showed that patient requests decreased the likelihood of antibiotic prescribing, while previous antibiotic prescribing behavior increased it. Clinical uncertainty did not have a significant effect on antibiotic prescribing.
Article
Health Care Sciences & Services
Sophie Stukas, George Goshua, Angus Kinkade, Rebecca Grey, Gregory Mah, Catherine M. Biggs, Shahin Jamal, Sonny Thiara, Tim T. Y. Lau, Jolanta Piszczek, Nilu Partovi, David D. Sweet, Agnes Y. Y. Lee, Cheryl L. Wellington, Mypinder S. Sekhon, Luke Y. C. Chen
Summary: This study compared the physiological responses and clinical outcomes of different doses of tocilizumab and dexamethasone in COVID-19 patients. The findings showed that both doses of tocilizumab effectively reduced inflammation and had similar 28-day mortality. In terms of cost-effectiveness, providing a fixed dose of 400 mg tocilizumab to all patients yielded comparable net monetary benefits to providing 8 mg/kg to half the patients.
LANCET REGIONAL HEALTH-AMERICAS
(2022)
Letter
Infectious Diseases
Caroline Spaner, Mariam Goubran, Audi Setiadi, Luke Y. C. Chen
LANCET INFECTIOUS DISEASES
(2022)
Article
Public, Environmental & Occupational Health
Sophie Y. Wang, Chiara Seghieri, Milena Vainieri, Oliver Groene
Summary: In the Tuscany region of Italy, the COVID-19 pandemic has resulted in significant reductions in hospitalization rates, especially for non-COVID-19 patients. The implementation of pandemic-related measures and fear of infection have contributed to these decreases, impacting overall hospitalization rates and specific acute medical conditions.
INTERNATIONAL JOURNAL OF PUBLIC HEALTH
(2022)
