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Quoted phrase not found in phrase index: "Familial hemophagocytic lymphohistiocytosis type 1"
Page 1
T-cell activation profiles distinguish hemophagocytic lymphohistiocytosis and early sepsis.
Chaturvedi V, Marsh RA, Zoref-Lorenz A, Owsley E, Chaturvedi V, Nguyen TC, Goldman JR, Henry MM, Greenberg JN, Ladisch S, Hermiston ML, Jeng M, Naqvi A, Allen CE, Wong HR, Jordan MB. Chaturvedi V, et al. Blood. 2021 Apr 29;137(17):2337-2346. doi: 10.1182/blood.2020009499. Blood. 2021. PMID: 33512385 Free PMC article.
Hemophagocytic lymphohistiocytosis (HLH) is a fatal disorder of immune hyperactivation that has been described as a cytokine storm. ...Finally, we observed that a threshold of >7% CD38high/HLA-DR+ cells among CD8+ T cells had strong positive and negative predi …
Hemophagocytic lymphohistiocytosis (HLH) is a fatal disorder of immune hyperactivation that has been described as a cytokine s …
Recent advances and evolving concepts in Still's disease.
Ruscitti P, Cantarini L, Nigrovic PA, McGonagle D, Giacomelli R. Ruscitti P, et al. Nat Rev Rheumatol. 2024 Feb;20(2):116-132. doi: 10.1038/s41584-023-01065-6. Epub 2024 Jan 11. Nat Rev Rheumatol. 2024. PMID: 38212542 Review.
At the same time, phenotypic evidence indicates the involvement of autoinflammatory processes. Evidence also implicates the type I interferon signature, mechanistic target of rapamycin complex 1 signalling and ferritin in the pathogenesis of Still's disease and MAS. ...His …
At the same time, phenotypic evidence indicates the involvement of autoinflammatory processes. Evidence also implicates the type I in …
Characteristics and predictors of post-transplant-associated hemophagocytic lymphohistiocytosis in adults.
Hattori N, Sato M, Uesugi Y, Nakata A, Sasaki Y, Shimada S, Watanuki M, Fujiwara S, Kawaguchi Y, Arai N, Uto Y, Matsui T, Yanagisawa K, Tahara S, Koeffler HP, Iezumi K, Nakamaki T. Hattori N, et al. Int J Hematol. 2021 May;113(5):693-702. doi: 10.1007/s12185-020-03067-6. Epub 2021 Jan 1. Int J Hematol. 2021. PMID: 33385294
Hemophagocytic lymphohistiocytosis (HLH) is an uncontrolled hyperinflammatory disorder driven by an overactive immune system that results in high mortality. Post-transplant-associated hemophagocytic lymphohistiocytosis (PT-HLH) is a type of seco …
Hemophagocytic lymphohistiocytosis (HLH) is an uncontrolled hyperinflammatory disorder driven by an overactive immune system t …
Diagnostic and therapeutic caveats in Griscelli syndrome.
Castaño-Jaramillo LM, Lugo-Reyes SO, Cruz Muñoz ME, Scheffler-Mendoza SC, Duran McKinster C, Yamazaki-Nakashimada MA, Espinosa-Padilla SE, Saez-de-Ocariz Gutierrez MDM. Castaño-Jaramillo LM, et al. Scand J Immunol. 2021 Jun;93(6):e13034. doi: 10.1111/sji.13034. Epub 2021 Mar 20. Scand J Immunol. 2021. PMID: 33660295 Free article. Review.
Dermic granulomas and immunodeficiency with infectious and HLH predisposition have been described in GS type 2 (GS2). Neurologic alterations might be seen in GS type 1 (GS1) and GS type 2 (GS2), due to different mechanisms. ...
Dermic granulomas and immunodeficiency with infectious and HLH predisposition have been described in GS type 2 (GS2). Neurologic alte …
Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management.
Gholam C, Grigoriadou S, Gilmour KC, Gaspar HB. Gholam C, et al. Clin Exp Immunol. 2011 Mar;163(3):271-83. doi: 10.1111/j.1365-2249.2010.04302.x. Clin Exp Immunol. 2011. PMID: 21303357 Free PMC article. Review.
Familial haemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune dysregulation associated with uncontrolled T cell and macrophage activation and hypercytokinaemia. ...Type 2 is caused by mutations in the perforin (PRF1) gene …
Familial haemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune dysregulation associated w …
Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorders.
Park S, Ko YH. Park S, et al. J Dermatol. 2014 Jan;41(1):29-39. doi: 10.1111/1346-8138.12322. J Dermatol. 2014. PMID: 24438142 Review.
Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T-cell LPD of childhood. ...The clinical course of CAEBV disease-type T/NK LPD can be smoldering, persistent or progressive, depending on …
Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T-cell LPD …
STXBP2 mutations in children with familial haemophagocytic lymphohistiocytosis type 5.
Cetica V, Santoro A, Gilmour KC, Sieni E, Beutel K, Pende D, Marcenaro S, Koch F, Grieve S, Wheeler R, Zhao F, zur Stadt U, Griffiths GM, Aricò M. Cetica V, et al. J Med Genet. 2010 Sep;47(9):595-600. doi: 10.1136/jmg.2009.075341. J Med Genet. 2010. PMID: 20798128 Free PMC article.
BACKGROUND: Familial haemophagocytic lymphohistiocytosis (FHL) is a rare immune deficiency with uncontrolled inflammation; the clinical course usually starts within the first years of life, and is usually fatal unless promptly treated and then cured with haem …
BACKGROUND: Familial haemophagocytic lymphohistiocytosis (FHL) is a rare immune deficiency with uncontrolled inflammation; the …
Fatal COVID-19 Infection in Two Children with STAT1 Gain-of-Function.
Staines-Boone AT, Vignesh P, Tsumura M, de la Garza Fernández G, Tyagi R, Rawat A, Das J, Tomomasa D, Asano T, Hijikata A, Salazar-Gálvez Y, Kanegane H, Okada S, Reyes SOL. Staines-Boone AT, et al. J Clin Immunol. 2023 Dec 22;44(1):20. doi: 10.1007/s10875-023-01634-0. J Clin Immunol. 2023. PMID: 38129739
While SARS-CoV-2 infection causes a mild disease in most children, SARS-CoV-2 infection may be lethal in a few of them. In the defense against SARS-CoV-2, type I interferons are key players, and several studies have identified a defective or neutralized interferon response …
While SARS-CoV-2 infection causes a mild disease in most children, SARS-CoV-2 infection may be lethal in a few of them. In the defense again …
Genotype characteristics and immunological indicator evaluation of 311 hemophagocytic lymphohistiocytosis cases in China.
Zhang J, Sun Y, Shi X, Zhang R, Wang Y, Xiao J, Cao J, Gao Z, Wang J, Wu L, Wei W, Wang Z. Zhang J, et al. Orphanet J Rare Dis. 2020 May 6;15(1):112. doi: 10.1186/s13023-020-01390-z. Orphanet J Rare Dis. 2020. PMID: 32375849 Free PMC article.
BACKGROUND: Primary hemophagocytic lymphohistiocytosis (pHLH) is a genetic disorder that is classically diagnosed by genetic testing. ...Flow cytometry screening for deficient proteins, including perforin, SAP, and XIAP, showed a relatively high sensitivity (83.33-9 …
BACKGROUND: Primary hemophagocytic lymphohistiocytosis (pHLH) is a genetic disorder that is classically diagnosed by genetic t …
Hemophagocytic Lymphohistiocytosis in Patients With Primary Immunodeficiency.
Cetinkaya PG, Cagdas D, Gumruk F, Tezcan I. Cetinkaya PG, et al. J Pediatr Hematol Oncol. 2020 Aug;42(6):e434-e439. doi: 10.1097/MPH.0000000000001803. J Pediatr Hematol Oncol. 2020. PMID: 32324696
Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled and excessive immune responses with high mortality. ...The patients were evaluated in 2 groups including PID with hypopigmentation (n=7) (Chediak-Higashi syndrome [CHS] and Griscelli syndrome …
Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled and excessive immune responses with high mortality. …
88 results