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
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
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.
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
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
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
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
Multidisciplinary Sciences
Dominik Bursa, Agnieszka Bednarska, Andrzej Pihowicz, Marcin Paciorek, Andrzej Horban
Summary: This study assessed the occurrence of HLH features in patients with sepsis, finding that some patients may develop sepsis with HLH-like syndrome (SHLS) without impacting their risk of death. Duopenia, hypertriglyceridemia, hypofibrinogenemia, and high levels of sCD25 are uncommon in sepsis and may indicate real HLH syndrome. Hyperferritinemia, even as high as in real HLH syndrome, can be observed in sepsis patients.
SCIENTIFIC REPORTS
(2021)
Review
Gastroenterology & Hepatology
Elliot S. Coburn, Corey A. Siegel, Michael Winter, Eric D. Shah
Summary: HLH is rare among IBD patients treated with biologics, with most cases having identifiable infection or malignancy at diagnosis and a history of immunomodulator use. Survival rate is 70%, with risk factors possibly including younger age, male gender, Crohn's disease, and treatment induction phase.
DIGESTIVE DISEASES AND SCIENCES
(2021)
Article
Rheumatology
S. D. Good, S. D. Wade, V. C. Kyttaris
Summary: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening inflammatory syndrome that can be triggered by autoimmune diseases, malignancy, or infection. This study aimed to compare the clinical features and laboratory parameters of macrophage activation syndrome (MAS) and malignancy-associated HLH (mHLH) patients. Significant differences were found in platelet counts, soluble IL-2R concentration, and hepatomegaly between the two groups.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Pediatrics
Oksana Boyarchuk, Olha Dyvonyak, Tetyana Hariyan, Alla Volokha
Summary: This article reports a 3-year-old boy with APECED who had recurrent COVID-19 and developed retinopathy and autoimmune hepatitis after the first episode of SARS-CoV-2 infection. Subsequent infections triggered severe hyperinflammation with signs of HLH. Despite treatment, the patient had a fatal outcome. This article highlights the importance of recognizing and managing HLH in patients with immune dysregulation and impaired viral response.
FRONTIERS IN PEDIATRICS
(2023)
Article
Rheumatology
Maria L. Taylor, Kacie J. Hoyt, Joseph Han, Leslie Benson, Siobhan Case, Mia T. Chandler, Margaret H. Chang, Craig Platt, Ezra M. Cohen, Megan Day-Lewis, Fatma Dedeoglu, Mark Gorman, Jonathan S. Hausmann, Erin Janssen, Pui Y. Lee, Jeffrey Lo, Gregory P. Priebe, Mindy S. Lo, Esra Meidan, Peter A. Nigrovic, Jordan E. Roberts, Mary Beth F. Son, Robert P. Sundel, Maria Alfieri, Jenny Chan Yeun, Damilola M. Shobiye, Barbara Degar, Joyce C. Chang, Olha Halyabar, Melissa M. Hazen, Lauren A. Henderson
Summary: Implementing an evidence-based guideline in the management of hemophagocytic lymphohistiocytosis and macrophage activation syndrome in children improves clinical outcomes, including reduction in mortality, shorter time to decrease in inflammatory markers, and improved treatment effects.
JOURNAL OF RHEUMATOLOGY
(2022)
Review
Oncology
Jonathan Paolino, Nancy Berliner, Barbara Degar
Summary: HLH is a syndrome of multiorgan system dysfunction caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A common finding and diagnostic criterion of HLH is cytopenias in >= 2 cell lines. The mechanism of cytopenias in HLH is multifactorial, mainly driven by suppression of hematopoiesis by pro-inflammatory cytokines.
FRONTIERS IN ONCOLOGY
(2022)
Review
Rheumatology
Mehmet Soy, Pamir Atagunduz, Isik Atagunduz, Gulsan Turkoz Sucak
Summary: Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is an acute systemic inflammatory disorder characterized by excessive cytokine production and hyperferritinemia. It can be primary, caused by genetic mutations, or secondary, triggered by underlying disorders like malignancies or infections. In COVID-19 patients, secondary HLH and cytokine storm may lead to severe symptoms such as progressive fever, cytopenia, ARDS, and organ failure. Early differentiation and classification are crucial for appropriate treatment strategies.
RHEUMATOLOGY INTERNATIONAL
(2021)
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
Hematology
Alison M. Schram, Federico Campigotto, Ann Mullally, Annemarie Fogerty, Elena Massarotti, Donna Neuberg, Nancy Berliner
Review
Hematology
Alison M. Schram, Nancy Berliner
Editorial Material
Hematology
Nathan T. Connell, Nancy Berliner
Review
Hematology
Christopher Gibson, Nancy Berliner
Article
Hematology
Nathan T. Connell, Nancy Berliner
Editorial Material
Hematology
Nancy Berliner, Thomas D. Coates
Article
Hematology
Paul La Rosee, AnnaCarin Horne, Melissa Hines, Tatiana von Bahr Greenwood, Rafal Machowicz, Nancy Berliner, Sebastian Birndt, Juana Gil-Herrera, Michael Girschikofsky, Michael B. Jordan, Ashish Kumar, Jan A. M. van Laar, Gunnar Lachmann, Kim E. Nichols, Athimalaipet Ramanan, Yini Wang, Zhao Wang, Gritta Janka, Jan-Inge Henter
Article
Hematology
Mounica Vallurupalli, Nancy Berliner
Editorial Material
Medicine, Research & Experimental
Robert M. Stern, Nancy Berliner
JOURNAL OF CLINICAL INVESTIGATION
(2019)
Editorial Material
Hematology
Nancy Berliner, Andrew Roberts
Editorial Material
Hematology
Catherine Bollard, Nancy Berliner
Article
Hematology
Rebecca L. Zon, Nancy Berliner
Summary: Neutrophil abnormalities, such as neutrophilia and neutropenia, can have various causes including infection, medication, and congenital disorders. Evaluating the timing of the change and the patient's historical neutrophil count is crucial in diagnosing these abnormalities.
Editorial Material
Hematology
Nancy Berliner
Article
Rheumatology
Kathleen M. M. Vanni, Nancy Berliner, Nina P. Paynter, Robert J. Glynn, Jean MacFadyen, Joshua Colls, Fengxin Lu, Chang Xu, Paul M. Ridker, Daniel H. Solomon
ACR OPEN RHEUMATOLOGY
(2020)
