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)
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)
Review
Biochemistry & Molecular Biology
Raymond Chu, Charmaine van Eeden, Sneha Suresh, Wendy I. Sligl, Mohammed Osman, Jan Willem Cohen Tervaert
Summary: The COVID-19 pandemic caused by SARS-CoV-2 has raised concerns about a cytokine storm similar to HLH, questioning the effectiveness of current diagnostic criteria. More research is needed to confirm the existence of COVID-19 associated HLH and develop better methods for identification.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Engineering, Biomedical
Honglan Wang, Huiwen Liu, Jia Li, Chunying Liu, Hui Chen, Junying Li, Chunyan Sun, Tao Guo, Zhiqing Pang, Bo Zhang, Yu Hu
Summary: LMNP, a novel type of macrophage membrane-coated nanoparticles, can effectively dampen HLH and alleviate related symptoms. This study provides alternative insights for the treatment of other cytokine storm-mediated pathologic conditions.
BIOACTIVE MATERIALS
(2023)
Article
Allergy
Yuting Tang, Qian Xu, Hui Luo, Xiaomei Yan, Gaoxiang Wang, Liang Hu, Jin Jin, David P. Witte, Rebecca A. Marsh, Liang Huang, Gang Huang, Jianfeng Zhou
Summary: This study found that the collaborative roles of IL-10 and IL-18 can drive a hyperinflammatory syndrome similar to hemophagocytic lymphohistiocytosis (HLH). The study also discovered that blocking IL-10 can be protective in patients with HLH.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Immunology
Kristina E. N. Clark, William D. Nevin, Tabitha Mahungu, Helen Lachmann, Animesh Singh
Summary: This study found similarities in the clinical manifestations and molecular inflammatory responses between COVID-19 patients and HLH patients, but did not support the use of HLH risk-scoring methodology for risk stratification in COVID-19 patients.
CLINICAL INFECTIOUS DISEASES
(2021)
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
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
Endocrinology & Metabolism
C. Scozzafava, S. Sestito, D. Concolino, A. Giannotta, M. Miraglia Del Giudice, G. F. Parisi, G. Ceravolo, C. Cuppari, L. Pensabene, D. Sutera, A. Spagnuolo, R. Gallizzi
Summary: Conditions such as HLH, MAS, sepsis, and MIS-C share similarities in clinical and laboratory aspects, including elevated ferritin levels. Therefore, in pediatric emergency settings, high ferritin levels accompanied by fever should prompt consideration of these rare syndromes for timely therapy.
JOURNAL OF BIOLOGICAL REGULATORS AND HOMEOSTATIC AGENTS
(2022)
Review
Immunology
Camille Keenan, Kim E. Nichols, Sabrin Albeituni
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome driven by overactive T cells and macrophages that abundantly secrete pro-inflammatory cytokines. Etoposide-based regimens have reduced mortality associated with HLH, but the 5-year survival rate remains low. Novel cytokine-directed therapies, such as ruxolitinib, are being studied to improve outcomes in HLH.
FRONTIERS IN IMMUNOLOGY
(2021)
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
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
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.
Article
Medicine, General & Internal
Sachin Gautam, Gaurav Sharma, Sumeet Singla, Sandeep Garg
Summary: This article presents a case of secondary hemophagocytic lymphohistiocytosis (sHLH) occurring after recovery from COVID-19. It highlights the importance of surveillance for sHLH in the post-COVID-19 phase.
FRONTIERS IN MEDICINE
(2022)
Article
Medicine, General & Internal
Sebastian Schnaubelt, Daniel Tihanyi, Robert Strassl, Ralf Schmidt, Sonja Anders, Anton N. Laggner, Hermine Agis, Hans Domanovits
Summary: The study reviewed three patients with COVID-19 who exhibited hyperinflammation and cytokine storm, requiring intensive care treatment. Diagnosis of secondary sHLH was made using HScore. A treatment regimen of methylprednisolone, pentaglobin, and anakinra was developed and administered. One patient survived while two others, diagnosed late, deceased.
Article
Rheumatology
Emre Tekgoz, Seda Colak, Kubra G. Yardimci, Orhan Kucuksahin, Muhammet Cinar, Sedat Yilmaz, Timucin Kasifoglu, Cemal Bes, Burcu Yagiz, Abdulsamet Erden, Levent Kilic, Nilufer A. Kanitez, Ali Ertenli, Belkis N. Coskun, Elif D. Ediboglu, Ridvan Mercan, Sedat Kiraz, Veli Yazisiz, Omer Karadag, Pamir Atagunduz, Umut Kalyoncu
Summary: This study investigated the preferences of physicians in using biological disease-modifying antirheumatic drugs (bDMARDs) for rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy. The study found that rituximab was the most frequently prescribed bDMARD for RA patients, while secukinumab and etanercept were the preferred choices for SpA patients. The interval between malignancy diagnosis and starting bDMARD treatment was less than 60 months for a majority of patients.
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
(2022)
Review
Rheumatology
Mehmet Soy, Gokhan Keser, Pamir Atagunduz, Melek Yalcin Mutlu, Alper Gunduz, Gizem Koybasi, Cemal Bes
Summary: The article discusses the importance of COVID-19 vaccination in AIIRD patients and provides precautions to be taken before vaccination, including vaccination when disease activity is under control and before the use of biological DMARDs.
CLINICAL RHEUMATOLOGY
(2021)
Article
Ophthalmology
Nazife Sule Yasar Bilge, Umut Kalyoncu, Pamir Atagunduz, Ediz Dalkilic, Yavuz Pehlivan, Orhan Kucuksahin, Cemal Bes, Servet Akar, Muhammet Cinar, Hakan Emmungil, Duygu Ersozlu, Askin Ates, Ridvan Mercan, Gezmis Kimyon, Suleyman Serdar Koca, Emel Gonullu, Veli Yazisiz, Emre Tekgoz, Nilufer Alpay-Kanitez, Abdulsamet Erden, Sedat Kiraz, Belkis Nihan Coskun, Burcu Yagiz, Ufuk Ilgen, Omer Karadag, Levent Kilic, Ihsan Ertenli, Timucin Kasifoglu
Summary: The study aimed to analyze the frequency and factors associated with acute anterior uveitis (AAU) in different forms of Spondyloarthritis (SpA) in a large Turkish cohort. It was found that older age, smoking history, delayed diagnosis, longer disease duration, arthritis, positive HLA-B27, family history of SpA, and radiographic damage were associated with a higher prevalence of uveitis in SpA patients. Psoriasis and psoriatic arthritis patients had a lower prevalence of uveitis. Early detection of uveitis can aid in the diagnosis of SpA.
AMERICAN JOURNAL OF OPHTHALMOLOGY
(2021)
Review
Immunology
Pamir Ataguenduez, Goekhan Keser, Mehmet Soy
Summary: New variants of SARS-CoV-2 have raised concerns about the spread of COVID-19, particularly among patients with autoimmune/autoinflammatory rheumatic diseases (AIIRD). The timing of COVID-19 vaccination in AIIRD patients using Interleukin-1 (IL-1) blockers is still a topic of debate.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Ilkay Ergenc, Haluk Tarik Kani, Cemal Aydin Gundogmus, Rabia Ergelen, Naime Afsar Satis, Gazanfer Ekinci, Mehmet Pamir Atagunduz
Summary: This study investigates the value of reporting acute inflammatory and structural changes of the sacroiliac joint on magnetic resonance enterography (MRE) as auxiliary findings to aid in the diagnosis of axial spondyloarthritis. The study finds that in addition to intestinal findings, reporting these changes on MRE can alert physicians to the presence of previously undiagnosed axial spondyloarthritis.
Article
Rheumatology
P. Atagunduz, S. Kiraz, S. Akar, O. Kucuksahin, A. Erden, A. Aksoy, B. N. Coskun, B. Yagiz, C. Bes, N. Alpay Kanitez, L. Kilic, O. Karadag, T. Kasifoglu, H. Emmungil, M. Cinar, G. Kimyon, V. Yazisiz, A. Ates, D. Ersozlu, E. Gonullu, R. Mercan, I. Ertenli, U. Kalyoncu
Summary: The clinical and laboratory factors associated with bamboo spine were analyzed in this study. The results showed that bamboo spine was more common in males and associated with delayed diagnosis, high BMI, hip involvement, and enthesitis.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Letter
Rheumatology
S. Celik, M. E. Kutu, C. Bal, B. Karadeniz, P. Atagunduz, M. Soy
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Review
Medicine, General & Internal
Gokhan Keser, Pamir Atagunduz, Mehmet Soy
Summary: LVV, including TAK and GCA, causes granulomatous vascular inflammation mainly in large vessels. The best way to diagnose LVV is to combine medical history, physical examination, various laboratory tests, and imaging modalities.
POLISH ARCHIVES OF INTERNAL MEDICINE-POLSKIE ARCHIWUM MEDYCYNY WEWNETRZNEJ
(2022)
Article
Rheumatology
Ufuk Ilgen, Omer Karadag, Hakan Emmungil, Orhan Kucuksahin, Suleyman Serdar Koca, Abdulsamet Erden, Cemal Bes, Nilufer Alpay Kanitez, Ediz Dalkilic, Servet Akar, Ridvan Mercan, Muhammet Cinar, Timucin Kasifoglu, Emel Gonullu, Gezmis Kimyon, Duygu Ersozlu, Pamir Atagunduz, Levent Kilic, Ihsan Ertenli, Veli Yazisiz, Askin Ates, Sedat Kiraz, Umut Kalyoncu
Summary: This study compared TST and QFT-GIT tests in RA and SpA patients scheduled for biological and targeted synthetic DMARDs. The results showed that TST positivity was more pronounced in SpA compared to RA, and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cutoff for both diseases could result in overestimating LTBI in SpA.
RHEUMATOLOGY INTERNATIONAL
(2022)
Review
Biology
Mehmet Soy, Gokhan Keser, Pamir Atagunduz
Summary: COVID-19 is a viral infection caused by SARS-CoV-2, which can lead to a hyperinflammatory state known as COVID-CS. The pathogenesis of COVID-CS may involve insufficient viral clearance and excessive cytokine response, while the diagnosis relies on clinical symptoms and findings. Management includes antiviral agents and supportive care.
TURKISH JOURNAL OF BIOLOGY
(2021)