Results 71 to 80 of about 36,076 (253)

Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin

Advances in Rheumatology
Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes ...
F. Sztajnbok, A. Fonseca, Leonardo Rodrigues Campos, Kátia Lino, M. Rodrigues, R. M. Silva, R. D. de Almeida, S. Perazzio, M. Carvalho   +8 more
semanticscholar   +1 more source

Hemophagocytic Lymphohistiocytosis and Infection: A Literature Review

Cureus, 2022
Hemophagocytic lymphocytosis (HLH) is a life-threatening, underdiagnosed syndrome caused by the excessive release of inflammatory mediators. Primary lymphocytosis is usually seen in young children and is associated with genetic defects, while secondary ...
Evgenia Koumadoraki, Nikolaos Madouros, Shayka Sharif, Amber Saleem, S. Jarvis, Safeera Khan   +5 more
semanticscholar   +1 more source

Development and validation of a predictive model for diagnosing EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis

BMC Cancer
Purpose This study aims to identify distinguishing factors between EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis and non-neoplastic EBV-associated hemophagocytic lymphohistiocytosis.
Yuhong Yin, Wenzhi Zhang, Lizhen Zhao, Ying Li, Minchun Huang, Yu Han, Xiaoyan Wu   +6 more
doaj   +1 more source

Diffuse large B-cell lymphoma in an adolescent female presenting with Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis: a case report

Journal of Medical Case Reports, 2012
Introduction Hemophagocytic lymphohistiocytosis is characterized by multisystem inflammation, resulting from prolonged and intense activation of macrophages, histiocytes and CD8+ T-cells. Due to its variable presentation and non-specific findings, timely
Altaf Sadaf, Atreaga Grace M, Joshi Avni Y, Rodriguez Vilmarie   +3 more
doaj   +1 more source

Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective

Experimental and Molecular Medicine
Secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (sHLH/MAS) is a life-threatening immune disorder triggered by rheumatic disease, infections, malignancies, or medications.
T. T. Nguyen, Yoon Tae Kim, Geunyeol Jeong, Mirim Jin   +3 more
semanticscholar   +1 more source

Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure

Frontiers in Oncology, 2022
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of multiorgan system dysfunction that is caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A nearly ubiquitous finding and a diagnostic criterion of HLH
J. Paolino, N. Berliner, B. Degar
semanticscholar   +1 more source

Humanized immune system animal models and their recent applications

Animal Models and Experimental Medicine, EarlyView.
In this review, we provide an updated and comprehensive overview of the current state of humanized immune system animal model research. We discuss the variety of techniques used to produce humanized mice with their respective strengths and weaknesses, and evaluate the advantages and limitations of these models, including issues with incomplete immune ...
Nicolas Skuli, Rudra B. Amin, A’ishah Bakayoko, Marisa Kruidenier, Sahara Aqui, Sarah J. Skuli, Terence P. Gade   +6 more
wiley   +1 more source

Atypical familial hemophagocytic lymphohistiocytosis due to mutations in UNC13D and STXBP2 overlaps with primary immunodeficiency diseases

Haematologica, 2010
Background Familial hemophagocytic lymphohistiocytosis is a genetic disorder of lymphocyte cytotoxicity that usually presents in the first two years of life and has a poor prognosis unless treated by hematopoietic stem cell transplantation.
Jan Rohr, Karin Beutel, Andrea Maul-Pavicic, Thomas Vraetz, Jens Thiel, Klaus Warnatz, Ilka Bondzio, Ute Gross-Wieltsch, Michael Schündeln, Barbara Schütz, Wilhelm Woessmann, Andreas H. Groll, Brigitte Strahm, Julia Pagel, Carsten Speckmann, Gritta Janka, Gillian Griffiths, Klaus Schwarz, Udo zur Stadt, Stephan Ehl   +19 more
doaj   +1 more source

Interleukin‐18 Levels Are Associated With Disease Course in Patients With Still Disease Treated With Interleukin‐1 Inhibitors

Arthritis &Rheumatology, EarlyView.
Objective To evaluate the prognostic utility of circulating interleukin‐18 (IL‐18) levels in predicting disease activity, macrophage activation syndrome (MAS), and disease course in patients with Still disease (SD) receiving first‐line IL‐1 inhibitors (IL‐1i).
Matteo Trevisan, Manuela Pardeo, Ivan Caiello, Claudia Bracaglia, Arianna De Matteis, Valentina Matteo, Elena Loricchio, Fabrizio De Benedetti, Giusi Prencipe   +8 more
wiley   +1 more source

Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series

Journal of Medical Case Reports
Background Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory syndrome resulting from uncontrolled activation of T cells and macrophages, frequently leading to multiorgan failure.
Tianqi Xu, Ming Tang, Meiqing Wu, Xiaoyan Xue   +3 more
doaj   +1 more source