keyword
MENU ▼
Read by QxMD icon Read
search

HLH

keyword
https://www.readbyqxmd.com/read/28631531/interleukin-1-receptor-antagonist-anakinra-intravenous-immunoglobulin-and-corticosteroids-in-the-management-of-critically-ill-adult-patients-with-hemophagocytic-lymphohistiocytosis
#1
Philipp Wohlfarth, Hermine Agis, Guido A Gualdoni, Johannes Weber, Thomas Staudinger, Peter Schellongowski, Oliver Robak
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) causes multiple organ dysfunction frequently leading to intensive care unit (ICU) referral and/or death. We report on a series of critically ill adult patients treated with a non-etoposide-based regimen including interleukin 1 antagonist anakinra, intravenous immunoglobulin (IVIG), and/or corticosteroids (CS) for HLH. METHODS: Eight adult (≥18 years) ICU patients having received treatment with anakinra ± IVIG ± CS for HLH between March 2014 and March 2016 at a large tertiary care university hospital (Medical University of Vienna, Vienna, Austria) were retrospectively analyzed...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28629255/hemophagocytic-lymphohistiocytosis-associated-to-haemophilus-parainfluenzae-endocarditis-a-case-report
#2
D I Costescu Strachinaru, M Chaumont, D Gobin, L Sattar, M Strachinaru, E Karakike, A Roman, D Konopnicki
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that is characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. This syndrome is classified as primary (genetic) or secondary when acquired in the context of infections (usually viral), malignancies, rheumatologic and metabolic diseases. CASE SUMMARY: We report a case of HLH complicating a Haemophilus parainfluenzae mitral valve endocarditis and resolving under antibiotic and surgical treatment alone...
June 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28626191/hemophagocytic-lymphohistiocytosis-in-a-fatal-case-of-severe-fever-with-thrombocytopenia-syndrome
#3
Ayako Nakano, Hirohisa Ogawa, Yoshinori Nakanishi, Hiromi Fujita, Fumihiko Mahara, Kazuya Shiogama, Yutaka Tsutsumi, Toshiaki Takeichi
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging disease caused by a novel Bunyavirus with a high mortality rate. We herein report a fatal case of an 86-year-old woman with SFTS complaining of a fever, fatigue, and bicytopenia. Her condition deteriorated with rapid progression of bleeding tendency, disturbance of consciousness, and multiple organ failure leading to death on Day 6 of her illness. The histopathological findings in the autopsy revealed marked infiltration of macrophages with hemophagocytosis in the bone marrow, liver, and spleen leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28621800/a-consensus-review-on-malignancy-associated-hemophagocytic-lymphohistiocytosis-in-adults
#4
REVIEW
Naval Daver, Kenneth McClain, Carl E Allen, Sameer A Parikh, Zaher Otrock, Cristhiam Rojas-Hernandez, Boris Blechacz, Sa Wang, Milen Minkov, Michael B Jordan, Paul La Rosée, Hagop M Kantarjian
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. This review is focused on malignancy-associated HLH (M-HLH), in which possible mechanisms of pathogenesis include severe inflammation, persistent antigen stimulation by the tumor cells, and loss of immune homeostasis because of chemotherapy, hematopoietic stem cell transplantation, or infection...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28602651/a-bhlh-code-for-sexually-dimorphic-form-and-function-of-the-c-%C3%A2-elegans-somatic-gonad
#5
Maria D Sallee, Hana E Littleford, Iva Greenwald
How sexually dimorphic gonads are generated is a fundamental question at the interface of developmental and evolutionary biology [1-3]. In C. elegans, sexual dimorphism in gonad form and function largely originates in different apportionment of roles to three regulatory cells of the somatic gonad primordium in young larvae. Their essential roles include leading gonad arm outgrowth, serving as the germline niche, connecting to epithelial openings, and organizing reproductive organ development. The development and function of the regulatory cells in both sexes requires the basic-helix-loop-helix (bHLH) transcription factor HLH-2, the sole ortholog of the E proteins mammalian E2A and Drosophila Daughterless [4-8], yet how they adopt different fates to execute their different roles has been unknown...
June 19, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28601685/hematopoietic-stem-cell-transplantation-in-patients-with-gain-of-function-stat1-mutation
#6
Jennifer W Leiding, Satoshi Okada, David Hagin, Mario Abinun, Anna Shcherbina, Dmitry N Balashov, Vy H D Kim, Adi Ovadia, Stephen L Guthery, Michael Pulsipher, Desa Lilic, Lisa A Devlin, Sharon Christie, Mark Depner, Sebastian Fuchs, Annet van Royen-Kerkhof, Caroline Lindemans, Aleksandra Petrovic, Kathleen E Sullivan, Nancy Bunin, Sara Sebnem Kilic, Fikret Arpaci, Oscar de la Calle-Martin, Laura Martinez-Martinez, Juan Carlos Aldave, Masao Kobayashi, Teppei Ohkawa, Kohsuke Imai, Akihiro Iguchi, Chaim M Roifman, Andrew R Gennery, Mary Slatter, Hans D Ochs, Tomohiro Morio, Troy R Torgerson
BACKGROUND: Gain of function mutations in signal transducer and activator of transcription 1 (GOF-STAT1) cause a susceptibility to a range of infections, autoimmunity, immune dysregulation, and combined immunodeficiency. Disease manifestations can be mild or severe and life threatening. Hematopoietic stem cell transplantation (HSCT) has been utilized in some patients with more severe symptoms to treat and cure the disorder. The outcome of HSCT for this disorder is, however, not well established...
June 7, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28600178/a-novel-ghbee1-like-gene-of-cotton-causes-anther-indehiscence-in-transgenic-arabidopsis-under-uncontrolled-transcription-level
#7
Eryong Chen, Xiaoqian Wang, Qian Gong, Hamama Islam Butt, Yanli Chen, Chaojun Zhang, Zuoren Yang, Zhixia Wu, Xiaoyang Ge, Xianlong Zhang, Fuguang Li, Xueyan Zhang
Male-sterile lines are very important for selective breeding, and anther dehiscence defect is an effective way to generate male-sterile lines. Although several bHLH-family proteins in Arabidopsis have been characterized, little is known about the role of bHLH-family proteins in cotton. Here, we isolated a novel bHLH protein from cotton (Gossypium hirsutum), named GhBEE1-Like. Protein domain analysis showed that GhBEE1-Like contained a basic domain and an HLH domain. Subcellular localization analysis revealed that GhBEE1-Like was a nuclear-localized protein...
June 6, 2017: Gene
https://www.readbyqxmd.com/read/28589450/central-nervous-system-involvement-in-adults-with-haemophagocytic-lymphohistiocytosis-a-single-center-study
#8
Guilan Cai, Yini Wang, Xiaojing Liu, Yanfei Han, Zhao Wang
Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system (CNS). Neurological manifestations of HLH have been recognized in different studies with children, but they remain relatively ill-defined in adults with HLH. From March 2008 to October 2014, 289 adult patients with HLH were admitted to our center. Clinical, radiological, and cerebral spinal fluid (CSF) data of the patients with CNS involvement were reviewed, and a retrospective study in our single-center was carried out...
June 7, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28584842/diffuse-large-b-cell-lymphoma-with-secondary-hemophagocytic-lymphohistiocytosis-presenting-as-acute-liver-failure
#9
Ruchi Patel, Haren Patel, William Mulvoy, Sumit Kapoor
Hemophagocytic lymphohistiocytosis (HLH) and newly diagnosed malignant infiltration of liver are rare presentations of acute liver failure associated with poor prognosis. We report a case of a patient with acute liver failure caused by malignant infiltration by diffuse large B-cell lymphoma and secondary HLH.
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28584460/hemophagocytic-lymphohistiocytosis-associated-with-anaplasmosis
#10
Tamara M Johnson, Melinda S Brown, Mohamed Rabbat, Jihad Slim
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by unregulated macrophage and T-lymphocyte activation resulting in cytokine overproduction and subsequent histiocytic phagocytosis. Variant infections, particularly viruses have been postulated as the inciting factor for this potentially fatal disease. Herein, we will report a case of HLH associated with anaplasmosis.
April 2017: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/28582648/prognostic-factors-of-early-death-in-children-with-hemophagocytic-lymphohistiocytosis
#11
Ze-Bin Luo, Yuan-Yuan Chen, Xiao-Jun Xu, Ning Zhao, Yong-Min Tang
Hemophagocytic lymphohistiocytosis is a rapidly progressing and fatal disease. Early identification of early death for HLH patients based on the laboratory findings at the time of diagnosis could improve the overall survival. A retrospective study was performed on 95 Chinese pediatric patients with HLH. Patients' data including clinical features and laboratory findings at diagnosis were collected. In a multivariate Cox proportional hazard regression model analysis, albumin≤27.75g/L (hazard ratio (HR)=11.82, 95% confidence interval (CI) 2...
June 2, 2017: Cytokine
https://www.readbyqxmd.com/read/28575154/effects-of-propylene-glycol-or-elevated-lh-during-follicle-development-on-ovulation-fertilization-and-early-embryo-development
#12
Katherine S Hackbart, Robb W Bender, Paulo D Carvalho, Lais M Vieira, Ana R Dresch, Jerry N Guenther, Hidir Gencoglu, Anibal B Nascimento, Randy D Shaver, Milo C Wiltbank
Seventeen non-lactating Holstein cows were superovulated in a Latin-square designed experiment to determine effects of increased propylene glycol (PROP) and LH during antral follicle development on ovarian function, fertilization, and early embryo quality. PROP was orally drenched every 4 h for 7d to induce hyperinsulinemia and associated metabolic changes. LH concentrations were altered by increasing LH (3-fold) during last 2 d of superovulation. Treatment groups were: 1) Control-oral drenching with water plus low-LH preparation; 2) HLH-water plus high-LH preparation; 3) PROP-drenching with PROP plus low-LH; 4) PROP/HLH-PROP plus high-LH...
May 30, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/28573910/haploidentical-hematopoietic-stem-cell-transplantation-for-adult-patients-with-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis
#13
Zhihui Li, Yini Wang, Jingshi Wang, Jia Zhang, Zhao Wang
To assess the efficacy of haploidentical hematopoietic stem cell transplantation (HSCT) in adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), we retrospectively analyzed 30 cases that presented at our institution. At the time of HSCT, 20 patients (66.7%) had achieved a response after receiving HLH-94 or salvage therapies. All patients underwent myeloablative conditioning followed by peripheral blood HSCT from their related, haploidentical donors. Twenty-six patients (86...
June 2, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28572174/go-with-the-flow-perforin-and-cd107a-in-hlh
#14
Melissa R Hines, Kim E Nichols
No abstract text is available yet for this article.
June 1, 2017: Blood
https://www.readbyqxmd.com/read/28571517/mechanistic-insights-into-the-activity-of-ptf1-p48-pancreas-transcription-factor-1a-probing-the-interactions-levels-of-ptf1-p48-with-e2a-e47-transcription-factor-e2-alpha-and-id3-inhibitor-of-dna-binding-3
#15
Nishith Saurav Topno, Muthu Kannan, Ramadas Krishna
Ptf1-p48 (Pancreas specific transcription factor 1a) is transcription regulatory protein known for the activation of exocrine specific genes. Downregulation of its expression formulates early stages of pancreatic adenocarcinoma as deduced by its association with oncogenic bHLH (Basic Helix-Loop-Helix) protein ID3 (Inhibitor of DNA binding 3) protein whose overexpression induces cytoplasmic mislocalization of Ptf1-p48. The precise mechanism and/or functional role of Ptf1-p48in promoting pancreatic cancer is vague...
June 15, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28565867/hemophagocytic-lymphohistiocytosis-in-a-patient-with-human-immunodeficiency-virus-infection-a-case-report
#16
Yijun Nie, Zhanglin Zhang, Hong Wu, Lagen Wan
Hemophagocytic lymphohistiocytosis (HLH), also termed hemophagocytic syndrome, is a severe, life-threatening inflammatory condition that results from an excessive, prolonged and ineffective immune response. The syndrome occurs due to overactive macrophages from the bone marrow or lymph tissue that phagocytose erythrocytes leukocytes and platelets. HLH in a patient with human immunodeficiency virus infection has rarely been studied. The present case study described an uncommon case of this syndrome in combination with human immunodeficiency virus infection in a patient, who eventually succumbed to severe infection and multiple organ failure following the refusal of medical treatment...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28562515/secondary-hemophagocytic-lymphohistocytosis-in-a-child-with-brucellosis
#17
Esra Pekpak, Benhur Sirvan Cetin
Hemophagocytic lymphohistocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that is characterized by proliferation of histiocytes and hemophagocytosis in different organs. The diagnostic criteria include fever, hepatosplenomegaly, bicytopenia, high serum ferritin level, decreased natural killer cell activity, elevated soluble CD25 level, high serum fasting triglyceride level or low fibrinogen level, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. HLH can be classified as primary and secondary...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28553383/hemophagocytic-lymphohistiocytosis-a-diagnostic-conundrum
#18
Brittany Grzybowski, Vijay A Vishwanath
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment...
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28550411/isolation-characterization-and-expression-of-proto-oncogene-cmyc-in-large-yellow-croaker-larimichthys-crocea
#19
Yonghua Jiang, Kunhuang Han, Shihai Chen, Yilei Wang, Ziping Zhang
cMyc is a vital transcription factor that involves in the regulation of cell proliferation, growth, differentiation, and apoptosis. In the present study, cMyc in Larimichthys crocea (Lc-cMyc) was cloned and analyzed for investigating its function. The full-length cDNA of Lc-cMyc was 2089 bp encoding a 440-amino-acid protein (Lc-cMyc). Lc-cMyc had the characteristic helix-loop-helix-leucine-zipper (HLH-LZ) DNA-binding domain and highly conservative in evolution. The expression of Lc-cMyc was detected by quantitative real-time PCR (qRT-PCR) and in situ hybridization, respectively...
May 27, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/28548191/-hemophagocytic-lymphohistiocytosis-experience-in-27-patients
#20
Fernando Warley, Belén M Bonella, M Silvina Odstrcil-Bobillo, Victoria Otero, Gabriel Waisman, Gisela Bendelman, Diego Giunta, Verónica Peuchot, Catalina M Ungaro
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. MATERIAL AND METHODS: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed...
March 2017: Revista Médica de Chile
keyword
keyword
23588
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"