Review
Medical Laboratory Technology
Tanya Sajan Ponnatt, Cullen M. Lilley, Kamran M. Mirza
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of immune regulation that requires rapid diagnosis and aggressive management. Understanding the pathogenesis and early diagnosis of HLH plays a crucial role in determining patient outcome. HLH can be caused by genetic mutations or acquired factors, and proper management is essential for improving prognosis.
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
(2022)
Article
Immunology
Xiao-Jun Xu, Ze-Bin Luo, Hua Song, Wei-Qun Xu, Jan-Inge Henter, Ning Zhao, Meng-Hui Wu, Yong-Min Tang
Summary: This study aims to investigate the cytokine patterns of different subtypes of pediatric HLH and the role of cytokines in evaluating disease features. The results showed that there were differences in cytokine levels among different HLH subtypes, and IFN-gamma and IL-10/IFN-gamma ratio were useful tools for differentiating HLH subtypes. A four-quadrant model based on these parameters provided a simple evaluation of the HLH situation.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Pediatrics
Ellen Go, Mira van Veenendaal, Cedric Manlhiot, Rayfel Schneider, Brian W. McCrindle, Rae S. M. Yeung
Summary: This study describes the disease course of a small proportion of patients with both Kawasaki disease and systemic juvenile idiopathic arthritis, characterized by refractory Kawasaki disease, high prevalence of coronary artery dilatation, and shared immunopathology potentially linking the two conditions.
FRONTIERS IN PEDIATRICS
(2021)
Review
Medicine, General & Internal
Trym Fauchald, Bjorn Blomberg, Hakon Reikvam
Summary: This study reviewed reported cases of tuberculosis-associated hemophagocytic lymphohistiocytosis (HLH) in the English literature and summarized the epidemiology, diagnostics, treatment, and mortality in these cases. Among 116 patients with tuberculosis-associated HLH, there was a male:female ratio of about 3:2 and the age at presentation ranged from 12 days to 83 years. Most patients received both tuberculostatic and specific immunomodulating treatment, resulting in a higher survival rate compared to those receiving only one type of treatment. The overlapping symptoms between disseminated tuberculosis and HLH pose challenges in diagnosis and treatment, leading to increased mortality. Therefore, tuberculosis should be considered as a potential trigger of HLH, and appropriate investigations are needed for diagnosis and treatment.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Infectious Diseases
Marie-Lisa Hieber, Rosanne Sprute, Dennis A. Eichenauer, Michael Hallek, Ron D. Jachimowicz
Summary: This study reported a case of severe HLH after SARS-CoV-2 immunization and reviewed all reported cases of COVID-19 vaccine-associated HLH. It was found that COVID-19 vaccines may occasionally trigger HLH, and Anakinra may be an efficacious treatment option for this condition.
Article
Medicine, General & Internal
Clara Baverez, Maximilien Grall, Mathieu Gerfaud-Valentin, Sarah De Gail, Alexandre Belot, Thomas Perpoint, Emmanuelle Weber, Quitterie Reynaud, Pascal Seve, Yvan Jamilloux
Summary: Anakinra is an effective and safe treatment for secondary HLH, showing rapid symptom relief and reduction of inflammatory markers. Further prospective randomized clinical trials are needed to confirm its efficacy.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Rheumatology
Claas H. Hinze, Dirk Foell, Christoph Kessel
Summary: Systemic juvenile idiopathic arthritis (sJIA) is a disease characterized by severe systemic inflammation and arthritis. It poses challenges to rheumatologists treating pediatric and adult patients worldwide. Although treatment plans exist for classic sJIA, there is still a lack of clear treatment approaches for early sJIA without arthritis and complicated sJIA.
NATURE REVIEWS RHEUMATOLOGY
(2023)
Article
Hematology
Howard Lin, Brooks P. Scull, Baruch R. Goldberg, Harshal A. Abhyankar, Olive E. Eckstein, Daniel J. Zinn, Joseph Lubega, Jennifer Agrusa, Nader El Mallawaney, Nitya Gulati, Lisa Forbes, Ivan Chinn, Rikhia Chakraborty, Jessica Velasquez, Jordana Goldman, Dalia Bashir, Fong Lam, Eyal Muscal, Michael M. Henry, Jay N. Greenberg, Stephan Ladisch, Michelle L. Hermiston, Lauren K. Meyer, Michael Jeng, Ahmed Naqvi, Kenneth McClain, Trung Nguyen, Hector Wong, Tsz-Kwong Man, Michael B. Jordan, Carl E. Allen
Summary: HLH is characterized by pathologic immune activation, and prompt initiation of immune suppression is crucial for survival. Plasma biomarkers can differentiate HLH from other inflammatory conditions, with IFN-gamma signaling uniquely elevated in HLH.
Review
Medicine, General & Internal
Charlotte Girard-Guyonvarc'h, Mathilde Harel, Cem Gabay
Summary: This review summarizes the role and importance of IL-18 and IL-18BP in AOSD and sJIA. It is found that the imbalance of IL-18/IL-18BP is associated with the occurrence of these diseases, and treatment with recombinant IL-18BP shows potential therapeutic effects.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Pediatrics
Tingyan He, Yu Xia, Ying Luo, Jun Yang
Summary: This study aimed to evaluate the efficacy and potential adverse effects of JAKi in patients with systemic juvenile idiopathic arthritis (SJIA). The results showed that JAKi may be an alternative or adjuvant agent for patients with persistently active disease, glucocorticoid-related adverse reactions, or SJIA-MAS.
FRONTIERS IN PEDIATRICS
(2023)
Article
Virology
Hans Martin Orth, Andre Fuchs, Nadine Luebke, Bjoern-Erik Ole Jensen, Tom Luedde
Summary: We report an unusual case of recurrent Hemophagocytic lymphohistiocytosis (HLH) caused by Parvovirus B19 infection. The recurrent viremia triggered three episodes of hyperinflammation, two of which were clearly diagnosed as HLH. This case provides new insight into triggering mechanisms for HLH.
Article
Immunology
David X. Liu, Bapi Pahar, Timothy K. Cooper, Donna L. Perry, Huanbin Xu, Louis M. Huzella, Ricky D. Adams, Amanda M. W. Hischak, Randy J. Hart, Rebecca Bernbaum, Deja Rivera, Scott Anthony, Marisa St Claire, Russell Byrum, Kurt Cooper, Rebecca Reeder, Jonathan Kurtz, Kyra Hadley, Jiro Wada, Ian Crozier, Gabriella Worwa, Richard S. Bennett, Travis Warren, Michael R. Holbrook, Connie S. Schmaljohn, Lisa E. Hensley
Summary: In this study, the researchers found that rhesus monkeys infected with the Ebola virus exhibit pathophysiological features similar to hemophagocytic lymphohistiocytosis syndrome/macrophage activation syndrome. Therefore, regulating inflammation and immune function may provide an effective treatment for controlling the pathogenesis of acute Ebola virus disease.
JOURNAL OF INFECTIOUS DISEASES
(2023)
Article
Immunology
Sylvain Raimbault, Guillaume Monneret, Morgane Gossez, Fabienne Venet, Alexandre Belot, Franck Zekre, Solene Remy, Etienne Javouhey
Summary: In this study, the researchers examined the use of monocyte human leucocyte antigen (mHLA)-DR as a potential diagnostic marker for secondary HLH (sHLH). They found that levels of mHLA-DR were significantly higher in patients with sHLH compared to healthy children and those with septic shock. Additionally, mHLA-DR levels decreased after treatment initiation. The findings suggest that elevated mHLA-DR could aid in the diagnosis of sHLH and distinguish it from septic shock.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Pediatrics
Mikhail M. Kostik, Eugenia A. Isupova, Konstantin Belozerov, Tatyana S. Likhacheva, Evgeny N. Suspitsin, Rinat Raupov, Vera V. Masalova, Irina A. Chikova, Margarita F. Dubko, Olga V. Kalashnikova, Vyacheslav G. Chasnyk, Randy Q. Cron
Summary: This study provides evidence for the efficacy and safety of short-term increased doses (2-3 times normal) of canakinumab in treating MAS associated with sJIA. Further research on the efficacy and safety of increased doses of canakinumab for the treatment of MAS in children with sJIA is warranted.
FRONTIERS IN PEDIATRICS
(2022)
Article
Immunology
Antonia Pascarella, Claudia Bracaglia, Ivan Caiello, Alessia Arduini, Gian Marco Moneta, Marianna Nicoletta Rossi, Valentina Matteo, Manuela Pardeo, Fabrizio De Benedetti, Giusi Prencipe
Summary: This study found that untreated sHLH/MAS patients had higher levels of phosphorylated STAT1 in monocytes compared to glucocorticoid-treated patients, indicating a hyperreactivity to IFN gamma stimulation. These findings suggest that pSTAT1 levels may serve as a potential biomarker for early identification of sHLH/MAS.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Mirkamal Tolend, Thitiporn Junhasavasdikul, Randy Q. Cron, Emilio J. Inarejos Clemente, Thekla von Kalle, Christian J. Kellenberger, Bernd Koos, Elka Miller, Marion A. van Rossum, Rotraud K. Saurenmann, Lynn Spiegel, Jennifer Stimec, Marinka Twilt, Nikolay Tzaribachev, Shelly Abramowicz, Simone Appenzeller, Linda Z. Arvidsson, Saurabh Guleria, Jacob L. Jaremko, Eva Kirkhus, Tore A. Larheim, Arthur B. Meyers, Jyoti Panwar, Cory M. Resnick, Susan C. Shelmerdine, Brian M. Feldman, Andrea S. Doria
Summary: A DCE survey was used to determine the relative importance weights of items and grades of the JAMRIS-TMJ, showing high convergent validity with an unrestricted, holistic vignette ranking method.
ARTHRITIS CARE & RESEARCH
(2022)
Article
Rheumatology
Arianna De Matteis, Claudia Bracaglia, Denise Pires Marafon, Anna Lucia Piscitelli, Maria Alessio, Roberta Naddei, Francesca Orlando, Giovanni Filocamo, Francesca Minoia, Angelo Ravelli, Jessica Tibaldi, Rolando Cimaz, Achille Marino, Gabriele Simonini, Maria Vincenza Mastrolia, Francesco La Torre, Ilaria Tricarico, Francesco Licciardi, Davide Montin, Maria Cristina Maggio, Clotilde Alizzi, Giorgia Martini, Adele Civino, Romina Gallizzi, Alma Nunzia Olivieri, Francesca Ardenti Morini, Giovanni Conti, Fabrizio De Benedetti, Manuela Pardeo
Summary: This study used real-world data to evaluate the effectiveness and safety of canakinumab in Italian patients with sJIA. The results showed that canakinumab was effective in treating sJIA and had no serious adverse events. The history of MAS and higher number of active joints were associated with poorer treatment outcomes.
Article
Rheumatology
Marta Mazzoni, Angela Pistorio, Francesca Magnaguagno, Stefania Viola, Alessia Urru, Gian Michele Magnano, Angelo Ravelli, Clara Malattia
Summary: The study found that many JIA patients still have subclinical synovitis and bone marrow edema on MRI despite being in clinical remission. These subclinical inflammations may indicate the risk of disease flare and joint deterioration, which could have important implications for the management of the disease.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Daniel D. Reiff, Randy Q. Cron
Summary: This review provides an in-depth analysis of multisystem inflammatory disease in children (MIS-C) following COVID-19. It discusses the clinical findings, pathophysiology, imaging and laboratory studies, treatment algorithms, and disease outcomes. MIS-C can be challenging to diagnose due to its non-specific presentation, but accurate diagnosis can be achieved through common laboratory features, imaging findings, and historical clues, enabling appropriate treatment with therapies such as IVIG. Aggressive treatment leads to good outcomes, and longitudinal studies shed light on long-term cardiac sequelae and recovery.
CURRENT RHEUMATOLOGY REPORTS
(2022)
Editorial Material
Rheumatology
Melissa L. Mannion, Randy Q. Cron
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Clara Malattia, Lorenzo Chiarella, Miriam Sansone, Angela Pistorio, Claudio Lavarello, Manuela Carpaneto, Raffaele Ferri, Angelo Ravelli, Lino Nobili
Summary: This study investigates sleep quality in juvenile fibromyalgia syndrome (JFS) and its impact on the global burden of the disease. The results show that patients with JFS have longer sleep period time, increased wake after sleep onset, and lower N3 distribution index compared to healthy peers. Subjective poor sleep quality is related to widespread pain, symptom severity, depressive symptoms, fatigue, and symptom severity upon awakening. Sleep complaints are a key characteristic of JFS and have significant effects on pain and depression.
JOURNAL OF RHEUMATOLOGY
(2023)
Editorial Material
Immunology
Randy Q. Cron
Summary: In addition to the acute phase, a significant percentage of SARS-CoV-2 patients experience prolonged illness with varying symptoms. Longitudinal data collection on immunologic, inflammatory, and metabolic factors has allowed the development of a composite signature that can predict recovery.
Review
Medicine, General & Internal
Roberta Naddei, Ana Rebollo-Gimenez, Marco Burrone, Valentina Natoli, Silvia Rosina, Alessandro Consolaro, Angelo Ravelli
Summary: Juvenile psoriatic arthritis (JPsA) is a controversial subset of juvenile idiopathic arthritis (JIA), accounting for 1-7% of cases. There has been debate about whether JPsA should be classified as a distinct category within JIA. Studies have shown that children with JPsA can be divided into two subgroups based on their clinical features and genetic determinants. The ongoing discussion has prompted the revision of the current classification of JPsA.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Biology
Daniel D. Reiff, Mingce Zhang, Randy Q. Cron
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by uncontrolled inflammation and immune system activation. Primary HLH (pHLH) is caused by genetic mutations that lead to organ failure and can be fatal. Secondary HLH (sHLH) can be triggered by genetic mutations in certain genes. This study focused on the significance of a DOCK2 mutation in the pathogenesis of sHLH and its implications for diagnosis and treatment.
Review
Immunology
Pui Y. Lee, Randy Q. Cron
Summary: Cytokine storm syndromes (CSSs) are potentially fatal hyperinflammatory states characterized by persistent immune cell activation and excessive cytokine production. CSSs can be caused by genetic errors of immunity, infections, chronic inflammatory diseases, or malignancies. This review explores the biology of different types of CSSs, the involvement of immune pathways, the contribution of host genetics, and discusses treatment approaches.
JOURNAL OF IMMUNOLOGY
(2023)
Review
Pediatrics
Adele Civino, Federico Diomeda, Luca Giordano, Maria Beatrice Damasio, Sandra Perrone, Romina Gallizzi, Angelo Ravelli, Prisco Piscitelli, Maria Cristina Maggio
Summary: Intra- and juxta-articular osteoid osteomas are rare and can mimic inflammatory monoarthritis. The diagnostic delay can be long, and misdiagnosis is common, especially in unusual sites. Knowledge of the atypical presentations and pitfalls is crucial for accurate diagnosis.
Article
Pediatrics
Daniel D. Reiff, Randy Q. Cron
Summary: Cytokine storm syndrome (CSS) is a serious condition caused by severe systemic inflammation and immune activation. The use of cytokine-directed therapies, such as interleukin-1 receptor antagonist, has shown promising results in treating CSS in ECMO patients.
Article
Rheumatology
Bita Shakoory, Ashley Geerlinks, Marta Wilejto, Kate Kernan, Melissa Hines, Micol Romano, David Piskin, Angelo Ravelli, Rashmi Sinha, Daniel Aletaha, Carl Allen, Hamid Bassiri, Edward M. Behrens, Joseph Carcillo, Linda Carl, Winn Chatham, Jeffrey Cohen, Randy Q. Cron, Erik Drewniak, Alexei A. Grom, Lauren A. Henderson, Annacarin Horne, Michael B. Jordan, Kim E. Nichols, Grant Schulert, Sebastiaan Vastert, Erkan Demirkaya, Raphaela Goldbach-Mansky, Fabrizio de Benedetti, Rebecca A. Marsh, Scott W. Canna, HLH MAS Task Force
Summary: Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening hyperinflammatory syndromes that can develop in many inflammatory conditions. Early identification and management are crucial to prevent organ failure and mortality. This effort aimed to provide evidence-based guidance to assist clinicians in optimizing decision-making in the early stages of HLH/MAS.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Editorial Material
Rheumatology
Daniel D. Reiff, Randy Q. Cron
Article
Rheumatology
Bita Shakoory, Ashley Geerlinks, Marta Wilejto, Kate Kernan, Melissa Hines, Micol Romano, David Piskin, Angelo Ravelli, Rashmi Sinha, Daniel Aletaha, Carl Allen, Hamid Bassiri, Edward M. Behrens, Joseph Carcillo, Linda Carl, Winn Chatham, Jeffrey I. Cohen, Randy Q. Cron, Erik Drewniak, Alexei A. Grom, Lauren A. Henderson, Annacarin Horne, Michael B. Jordan, Kim E. Nichols, Grant Schulert, Sebastiaan Vastert, Erkan Demirkaya, Raphaela Goldbach-Mansky, Fabrizio de Benedetti, Rebecca A. Marsh, Scott W. Canna
Summary: This study aims to provide evidence-based and consensus-based guidance for clinicians in the early diagnosis, treatment, and monitoring of HLH/MAS. Through research, questionnaires, and expert opinions, 6 overarching statements and 24 specific points to consider were developed, covering various aspects including early recognition, diagnosis, treatment, and monitoring. The involvement of multidisciplinary expertise is crucial for timely intervention and management.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Oncology
Toma S. Omofoye, Anganile Kalinga, Ramapriya Ganti, Frank J. Minja, Timothy B. Rooney
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Prarthna V. Bhardwaj, Renuka Dulala, Senthil Rajappa, Chandravathi Loke
Summary: The management of breast cancers in India needs greater emphasis on awareness, early detection, standard pathologic testing, and cost-effective solutions. With standardized management, outcomes similar to those of developed countries can be expected. Additionally, clinical trials aimed at a more diverse population in LMICs like India should be undertaken.
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Victoria E. Forbes, Mary D. Chamberlin, Vincent Dusabejambo, Tim Walker, Steve P. Bensen, Norrisa Haynes, Kathryn Nunes, Veauthyelau Saint-Joy, Frederick L. Makrauer
Summary: Global health education needs to adapt to the challenges we face, such as inequities and pandemics. Virtual teaching models are effective in delivering global health education and improving outcomes through bidirectional learning and equitable partnerships.
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Tefta Isufaj Haliti, Ilir Hoxha, Rubena Mojsiu, Rohini Mandal, Goksu Goc, Kreshnike Dedushi Hoti
Summary: The IOTA Simple Rules have high diagnostic performance in distinguishing between benign and malignant adnexal masses, providing a reliable tool for early diagnosis of ovarian cancer.
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Cecilia Felix Penido Mendes de Sousa, Jared Pasetsky, Gustavo Nader Marta, Megan Kassick, Fabio Ynoe Moraes, Luqman K. Dad
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Mary D. Chamberlin, Dafina Ademi Islami, Shqiptar Demaci, Richard J. Barth Jr
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Review
Oncology
Jeta Bunjaku, Arber Lama, Tawanda Pesanayi, Jeton Shatri, Mary Chamberlin, Ilir Hoxha
Summary: The evidence suggests that lifestyle factors such as alcohol and coffee intake may increase the risk of lung cancer, while tea intake may have a protective effect. However, the quality of evidence is currently low and further research is needed.
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Mary Chamberlin, Christopher Booth, Gabriel A. Brooks, Achille Manirakiza, Fidel Rubagumya, Verna Vanderpuye
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Article
Oncology
Anne Christine Buteau, Alicia Castelo-Loureiro, Regina Barragan-Carrillo, Suyapa Bejarano, Alba J. Kihn-Alarcon, Enrique Soto-Perez-de-Celis
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
Review
Oncology
Ilir Hoxha, Fitim Sadiku, Lot Hoxha, Midhet Nasim, Marie Anne Christine Buteau, Krenare Grezda, Mary D. Chamberlin
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
(2024)
