Results 41 to 50 of about 13,346 (189)

Hemophagocytic lymphohistiocytosis in leprosy

Leprosy Review, 2015
A patient from Southeast Asia was diagnosed with systemic lupus erythematosus. One year later, she experienced exacerbation of skin lesions and was diagnosed with erythema nodosum leprosum. Upon treatment, the patient developed hemophagocytic lymphohistiocytosis with multi-organ failure and died from invasive fungal infection.
Liv R, Høyvoll, Yngvar, Fløisand, Hilde Lang, Orrem, Ragnar, Gunnarsson, Linn, Landrø, Trine, Brevig, Peter, Gaustad, Ingvild, Nordøy   +7 more
openaire   +2 more sources

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

Concurrence of hemophagocytic lymphohistiocytosis and small-cell lung cancer in bone marrow: A case report and literature review

SAGE Open Medical Case Reports, 2022
Hemophagocytic lymphohistiocytosis is a rare and almost universally fatal disease in adults. A 60-year-old female patient presented to our hospital with a 3-day history of weakness and anorexia.
Wenyan Xu, Zhenxing Guo
doaj   +1 more source

The IL‐10/IL‐6 Ratio and the Risk Score: Two Cytokines‐Based Predictors for Malignancy‐Associated Hemophagocytic Lymphohistiocytosis in Adults (M‐HLHa)

American Journal of Hematology, EarlyView.
ABSTRACT The predictive value of cytokines (CK) for malignancy‐associated adult hemophagocytic lymphohistiocytosis (M‐HLHa) remains uncertain. We evaluated a cytokine‐based Risk Score (RS) and the IL‐10/IL‐6 Ratio to predict M‐HLHa. Adult patients (n = 112) from the French HLH cohort (NCT02113917) with complete data for nine key HLH related CK measured
Coralie Bloch, Stephanie Chhun, Nanthara Sritharan, Marine Gil, Raphael Lhote, Marouane Boubaya, Olivier Lambotte, David Launay, Claire Larroche, Estibaliz Lazaro, Francois Liffermann, Marc Michel, Jean‐Marie Michot, Pierre Morel, Louis Terriou, Geoffrey Urbansk, Jean‐Francois Viallard, Morgane Cheminant, Felipe Suarez, Yves Lepelletier, Geneviève de Saint Basile, Olivier Hermine, the French HLH Study Group, Coralie Bloch, Jean Philippe Jais, Francois Liffermann, Pierre Morel, Morgane Cheminant, Felipe Suarez, Olivier Hermine, Fabrice Bonnet, Pascal Godemer, Damaj Gandhi, Olivier Fain, Olivier Lambotte, David Launay, Louis Terriou, Claire Larroche, Estibaliz Lazaro, Jean‐Francois Viallard, Francois Liffermann, Olivier Lortholary, Marc Michel, Jean‐Marie Michot, Pierre Morel, Frederic Pene, Antoinette Perlat, Geoffrey Urbanski   +47 more
wiley   +1 more source

Atypical Clinical Course of Griscelli Syndrome Type 2 With Primarily Neurologic Presentation and Adult‐Onset in a 46‐Year‐Old Male

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Griscelli Syndrome Type 2 (GS2) is a rare autosomal recessive disorder caused by pathogenic mutations in the RAB27A gene. Typically, it is characterized by cutaneous hypopigmentation, immunodeficiency, with or without neurological abnormalities secondary to hemophagocytic lymphohistiocytosis (HLH). Without treatment, GS2 often results in fatal
Dzhoy Papingi, Michael Kutsche, Helena Lichtenfeld, Fanny Kortüm, Angela Abicht, Laura Herrmann, Theresia Herget   +6 more
wiley   +1 more source

A Case of Multiple Mitochondrial Dysfunctions Syndrome 1 and Review of the Literature

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Multiple mitochondrial dysfunctions syndrome 1 (MMDS1, MIM #605711) due to NFU1 gene defects is an ultra‐rare autosomal recessive inborn error of metabolism associated with reduced function of NFU1 iron–sulfur cluster (ISC) scaffold protein.
Charles R. DiFalco, Aaron Williams, Claudia Soler‐Alfonso, Emily Waskow, Elizabeth Mizerik, Fernando Scaglia, Chaya N. Murali   +6 more
wiley   +1 more source

Hemophagocytic lymphohistiocytosis in adults [PDF]

Korean Journal of Anesthesiology, 2014
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by pathological immune activation leading to a severe hyperinflammatory condition and organ damage including fever, cytopenia, hepatitis, coagulopathy, and central nervous system damage [1,2].
Hyun Jeong Lee, Min Jeong Lee, Soo-Jeong Kim, Seung Ho Jeong, Jeongmin Kim   +4 more
openaire   +3 more sources

Hemophagocytic lymphohistiocytosis: a case series of a Brazilian institution

Revista Brasileira de Hematologia e Hemoterapia, 2014
Objective:To describe the clinical and laboratory presentation of hemophagocytic lymphohistiocytosis in children treated at a referral institution.Methods:A retrospective descriptive study was carried out of seven children diagnosed with hemophagocytic ...
Daniela Guimarães Rocha Ferreira, Paulo do Val Rezende, Mitiko Murao, Marcos Borato Viana, Benigna Maria de Oliveira   +4 more
doaj   +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

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source