Results 51 to 60 of about 20,151 (222)

Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis

Haematologica, 2019
Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological ...
Isao Tsuboi, Tomonori Harada, Yoko Hirabayashi, Shin Aizawa   +3 more
doaj   +1 more source

Repeated pulmonary nodules as the primary symptom of familial hemophagocytic lymphohistiocytosis in adults: a case report and review

Journal of International Medical Research, 2023
Pulmonary nodules are usually considered to be associated with malignant tumors and benign lesions, such as granuloma, pulmonary lymph nodes, fibrosis, and inflammatory lesions.
Lulu Zhang, Chuanchuan Dong, Qiannan Wu, Yupeng Li, Liting Feng, Yanqing Xing, Yangdou Dong, Le Liu, Xiaohui Li, Rujie Huo, Yanting Dong, Erjing Cheng, Xiaoyan Ge, Tian Xinrui   +13 more
doaj   +1 more source

Delayed Recognition of Maternal G6PD Heterozygous Status Across Prenatal and Newborn Care Interfaces

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Glucose‐6‐phosphate dehydrogenase (G6PD) deficiency is the most common red blood cell enzymatic disorder worldwide. Although many heterozygotes are asymptomatic, affected neonates have an increased risk for hyperbilirubinemia and related complications.
Mona M. Makhamreh, Ramiah R. Vickers, Cheryl Mensah, Wanda Dosal, Jennifer R. McKinney, April D. Adams   +5 more
wiley   +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

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

Peripheral Blood DNA Methylation Changes Precede Lymphoma Diagnosis in Primary Sjögren's Disease

Arthritis &Rheumatology, EarlyView.
Objective Primary Sjögren's disease (SjD) is a systemic autoimmune disease associated with an increased risk of lymphoma. The molecular mechanisms underlying lymphomagenesis remain poorly understood, and sensitive biomarkers for early identification of patients at high risk of developing lymphoma are lacking.
Hanna Lidberg, Cecilia Fugmann, Juliana Imgenberg‐Kreuz   +2 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

Persistent Interleukin‐18 Fuels Expansion of CD38+HLA‐DR+CD8+ T Cells in Still Disease and Macrophage Activation Syndrome

Arthritis &Rheumatology, EarlyView.
Objective Still disease (SD) is an autoinflammatory disorder characterized by remarkably high interleukin‐18 (IL‐18) levels. Increasing evidence suggests that adaptive immunity also contributes to its pathogenesis, particularly in refractory courses. Macrophage activation syndrome (MAS), one of SD's most severe complications, is associated with further
Greta Rogani, Remco G. A. Erkens, Alejandra Bodelón, Tim R. Mocking, Marein T. M. Putmans, Freya M. C. H. Huijsmans, Lyanne J. P. M. Sijbers, Aafke M. De Ligt, Maurice J. H. Van Haaren, Rianne C. Scholman, Noël M. M. Dautzenberg, Joyce I. Meesters‐Ensing, Martina Rossano, Laura Porretti, Stefan Nierkens, Francesca S. Minoia, Sebastiaan J. Vastert, Jorg van Loosdregt   +17 more
wiley   +1 more source

Perforin gene transfer into hematopoietic stem cells improves immune dysregulation in murine models of perforin deficiency [PDF]

, 2015
Defects in perforin lead to the failure of T and NK cell cytotoxicity, hypercytokinemia, and the immune dysregulatory condition known as familial hemophagocytic lymphohistiocytosis (FHL).
Montiel-Equihua, CA, Arumugam, P, Carmo, M, Risma, KA, Blundell, MP, Hontz, AE, Jordan, MB, Gaspar, HB, Malik, P, Thrasher, AJ, Tiwari, S, Schambach, A, Baum, C, Alonso-Ferrero, ME   +13 more
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