Results 51 to 60 of about 22,112 (212)

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

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

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

Efficacy, safety and cost‐effectiveness of CAR‐T therapy

British Journal of Clinical Pharmacology, EarlyView.
CAR T‐cells demonstrate high efficacy in blood cancers, including ALL, MM and DLBCL. Innovations target solid tumours despite challenges such as antigen escape. Combination therapies enhance the delivery and infiltration of CAR T cells. Toxicity, cost and resistance remain major barriers to clinical use.
Emina Karahmet Sher, Amina Džidić‐Krivić, Emma Pinjic, Nejra Selak, Kanita Omerbasic, Alexandar Chupin, Andrej Belancic, Almir Fajkic   +7 more
wiley   +1 more source

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

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. [PDF]

, 2017
Background Macrophage activation syndrome (MAS) is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to systemic juvenile idiopathic arthritis (sJIA).
Antón López, Jordi, Boom, V., Lahdenne, P., Quartier, Pierre, Ravelli, Angelo, Vastert, Sebastiaan J., Wulffraat, Nico   +6 more
core   +1 more source

Successfully performed video capsule endoscopy in an 8‐month‐old infant weighing 7.5 kg

JPGN Reports, EarlyView.
Abstract Video capsule endoscopy (VCE) is a well‐established diagnostic tool for examining the small bowel. Limited data exist on its use in infants. To our knowledge, we present the first detailed case of a successful PillCam®SB3‐VCE performed in an 8‐month‐old infant weighing 7.5 kg with suspected small bowel bleeding following allogeneic ...
Paul‐Christoph Zeisler, Robert Thimme, Ulrike Teufel‐Schaefer, Michael Schultheiß   +3 more
wiley   +1 more source

Diagnosis and Management of Prenatal Hereditary Pyropoikilocytosis

Prenatal Diagnosis, EarlyView.
ABSTRACT Hereditary pyropoikilocytosis (HPP) is a severe hemolytic anemia caused by variants in SPTA1, SPTB, and EPB41. These weaken horizontal interactions in the erythrocyte cytoskeleton, causing membrane fragmentation and splenic sequestration. It will readily cause fetal anemia and often hydrops fetalis. Prenatal diagnosis requires first ruling out
Connor Hartzell, Samantha Stover, Nora Gibson, Timothy Olson, James Connelly, Matthew Grace, Jennifer Andrews   +6 more
wiley   +1 more source

Incidence and Survival of Hemophagocytic Lymphohistiocytosis Over Two Decades: A Population‐Based Study

European Journal of Haematology, EarlyView.
ABSTRACT Adult hemophagocytic lymphohistiocytosis (HLH) is a rare, life‐threatening syndrome triggered by various conditions. A nationwide study of the incidence and outcomes of HLH in Denmark over 23 years (2000–2023) was performed. Adults (≥ 18 years) with HLH and triggering diseases were identified in the Danish National Patient Registry and/or the ...
Mads Okkels Birk Lorenzen, Martin Jensen, Rasmus Rask Kragh Jørgensen, Paw Jensen, Henrik Frederiksen, Dennis L. Hansen, Daniel El Fassi, Morten Nørgaard Andersen, Tarec Christoffer El‐Galaly   +8 more
wiley   +1 more source