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https://www.readbyqxmd.com/read/29226650/multiple-nonleukemic-myeloid-sarcoma-associated-hemophagocytic-lymphohistiocytosis-in-an-adult
#1
Jingwen Zhang, Yunxin Zeng, Xiangzhong Zhang, Wenxing Lai, Yuxin Chen, Ruozhi Xiao
BACKGROUND: Nonleukemic myeloid sarcoma (MS) occurs rarely. Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal condition. We report a rare case of nonleukemic MS associated with HLH. METHODS: Hematologic investigation, 18F-FDG PET/CT, bone marrow aspirate and biopsy, and lymph node biopsy were performed in a 25-year-old male patient. RESULTS: The patient was given a short-term treatment of etoposide and dexamethasone to control HLH...
November 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/29225252/reactivation-of-hemophagocytic-lymphohistiocytosis-triggered-by-antithymocyte-globulin
#2
Kensuke Matsuda, Kazuhiro Toyama, Takashi Toya, Masako Ikemura, Fumihiko Nakamura, Mineo Kurokawa
A 16-year-old boy with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) underwent allogeneic hematopoietic stem cell transplantation after conditioning with fludarabine, melphalan, total body irradiation, and rabbit antithymocyte globulin (ATG). A severe, persistent infusion reaction occurred after the initial administration of ATG. Investigations showed a rapid increase in the levels of liver enzymes and ferritin, and the reactivation of HLH was confirmed by marked hemophagocytosis in the bone marrow...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29221209/direct-hpv-e6-myc-interactions-induce-histone-modifications-pol-ii-phosphorylation-and-htert-promoter-activation
#3
Yiyu Zhang, Aleksandra Dakic, Renxiang Chen, Yuhai Dai, Richard Schlegel, Xuefeng Liu
Human Papillomavirus Viruses (HPVs) are associated with the majority of human cervical and anal cancers and 10-30% of head and neck squamous carcinomas. E6 oncoprotein from high risk HPVs interacts with the p53 tumor suppressor protein to facilitate its degradation and increases telomerase activity for extending the life span of host cells. We published previously that the Myc cellular transcription factor associates with the high-risk HPV E6 protein in vivo and participates in the transactivation of the hTERT promoter...
November 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29215415/epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-mimicking-lymphoma-on-fdg-pet-ct
#4
Qingqing Pan, Yaping Luo, Huanwen Wu, Yanru Ma, Fang Li
A 15-year-old boy with fever, pancytopenia, and hepatosplenomegaly was diagnosed as hemophagocytic lymphohistiocytosis (HLH). F-FDG PET/CT showed hypermetabolic foci in the liver, spleen, and bone marrow, as well as multiple FDG-avid lymph nodes, which were highly suggestive of lymphoma. Specimens from splenectomy depicted a large number of macrophages/histiocytes with hemophagocytosis of erythrocytes, without evidence of malignancy. Considering increased Epstein-Barr virus (EBV) DNA in peripheral blood and positive staining for EBV-encoded RNA in the spleen, EBV-associated HLH was confirmed...
December 5, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29209549/macrophage-activation-syndrome-a-report-of-two-cases-and-a-literature-review
#5
Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29207147/effect-and-mechanism-of-rnai-targeting-wwtr1-on-biological-activity-of-gastric-cancer-cells-sgc7901
#6
Yuan Li, Fang-Liang Yang, Chun-Fu Zhu, Li-Ming Tang
Gastric cancer (GC) is one of the most common malignancies in the world. It is essential to develop novel targets and therapeutic approaches for GC, which requires identification of novel functional molecules. WW‑domain containing transcription regulator 1 (WWTR1) may activate many transcriptional factors and exhibit an important role in the development of various tissues in mammals. The results of the present study demonstrated that mRNA and protein levels of WWTR1 are increased in GC tissues and cell lines...
December 5, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29200153/dengue-associated-hemophagocytic-lymphohistiocytosis-a-rare-complication-of-a-common-infection-in-singapore
#7
Kai-Qian Kam, Shui Yen Soh, Rajat Bhattacharyya
Hemophagocytic lymphohistiocytosis (HLH) can progress rapidly, often leading to multisystem organ failure and death. Prompt recognition of the syndrome and institution of appropriate treatment are crucial steps in improving the outcome. Dengue virus infection is not commonly known to be associated with secondary HLH. We present a case of a child with dengue fever who subsequently developed classical features of HLH. He was treated successfully with 4 weeks of steroid monotherapy instead of the multidrug therapy proposed in the HLH 2004 protocol...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29197292/expression-and-regulation-of-inhibitor-of-dna-binding-proteins-id1-id2-id3-and-id4-at-the-maternal-conceptus-interface-in-pigs
#8
Jisoo Han, Heewon Seo, Yohan Choi, Choongdeok Lee, Meong Il Kim, Yubyeol Jeon, Joohyeong Lee, Minsun Hong, Sang-Hwan Hyun, Eunsong Lee, Hakhyun Ka
Inhibitor of DNA binding (ID) proteins, ID1, ID2, ID3, and ID4 are transcriptional regulators that have a helix-loop-helix (HLH) domain but not a basic DNA binding domain. ID proteins inhibit the functions of basic HLH transcription factors and regulate cell proliferation and differentiation. However, the expression and function of ID1, ID2, ID3, and ID4 at the maternal-conceptus interface are not fully understood in pigs. Therefore, we determined the expressions of ID1, ID2, ID3, and ID4 in porcine endometrium, conceptus, and chorioallantoic tissues...
November 29, 2017: Theriogenology
https://www.readbyqxmd.com/read/29194292/fetal-splenomegaly-a-review
#9
Marta Moreira, Rafael Brás, Daniela Gonçalves, Inês Alencoão, Gonçalo Inocêncio, Maria Rodrigues, Jorge Braga
Enlargement of the fetal spleen is usually found secondary to systemic diseases and is frequently associated with hepatomegaly. By far, the most common causes of fetal splenomegaly are infectious. Other etiologies responsible for this sign are hemolytic anemia, congestive cardiac failure, metabolic disorders, and rarely, leukemia, lymphoma, and histiocytosis.We report a case of prenatal splenomegaly diagnosed at 35 weeks, confirmed in the postnatal period. The postnatal workup showed the newborn had a familial type 3 form of hemophagocytic lymphohistiocytosis (HLH)...
November 29, 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/29187030/hemophagocytic-lymphohistiocytosis-secondary-to-t-cell-histiocyte-rich-large-b-cell-lymphoma-in-an-adolescent-male
#10
Jonathan L Metts, Sunita I Park, Michael A Briones, Frank G Keller
Hemophagocytic Lymphohistiocytosis (HLH) is a hyperinflammatory disorder that may be encountered as a primary or secondary phenomenon. HLH secondary to lymphoma has been described, more frequently in adults than in children. T-cell/Histiocyte-rich B-cell lymphoma (THRLBCL) is a large B-cell lymphoma that resides in a microenvironment of robust host immune response and has previously been associated with HLH in adults. Here, we describe the first case of HLH secondary to THRLBCL in an adolescent patient.
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29181134/a-nine-month-old-boy-with-atypical-hemophagocytic-lymphohistiocytosis
#11
Monia Ouederni, Monia Ben Khaled, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, Mohamed Bejaoui
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a wide range of infectious and auto-immune tests to be performed. After an extensive diagnostic workup, he was referred to the immune-hematologic unit for HLH suspicion with an unknown cause...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29178470/lymphocyte-independent-pathways-underlie-the-pathogenesis-of-murine-cytomegalovirus-associated-secondary-hemophagocytic-lymphohistiocytosis
#12
Brisse Ellen, Imbrechts Maya, Mitera Tania, Vandenhaute Jessica, Berghmans Nele, Boon Louis, Wouters H Carine, Snoeck Robert, Andrei Graciela, Matthys Patrick
Hemophagocytic lymphohistiocytosis (HLH) constitutes a spectrum of immunological disorders characterized by uncontrolled immune activation and key symptoms such as fever, splenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia and hepatitis. In genetic or primary HLH, hyperactivated CD8+ T cells are the main drivers of pathology. However, in acquired, secondary HLH, the role of lymphocytes remains vague. In the present study the involvement of lymphocytes in the pathogenesis of a cytomegalovirus-induced model of secondary HLH was explored...
November 27, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29178359/elevated-luteinising-hormone-despite-normal-testosterone-levels-in-older-men-natural-history-risk-factors-and-clinical-features
#13
Robert J A H Eendebak, Tomas Ahern, Agnieszka Swiecicka, Stephen R Pye, Terence W O'Neill, Bartfai Gyorgy, Felipe F Casanueva, Mario Maggi, Gianni Forti, Aleksander Giwercman, Thang S Han, Jolanta Słowikowska-Hilczer, Michael E J Lean, Margus Punab, Neil Pendleton, Brian G Keevil, Dirk Vanderschueren, Martin K Rutter, Gindo Tampubolon, Royston Goodacre, Ilpo T Huhtaniemi, Frederick C W Wu
OBJECTIVE: Elevated LH with normal testosterone (T) suggests compensated dysregulation of the gonadal axis. We describe the natural history, risk factors and clinical parameters associated with the development of high LH (HLH, LH>9.4 U/L) in ageing men with normal T (T≥10.5 nmol/L). DESIGN, PATIENTS AND MEASUREMENTS: We conducted a 4.3 year prospective observational study of 3,369 community-dwelling European men aged 40-79 years. Participants were classified as: incident (i) HLH (n=101, 5...
November 27, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29177266/atypical-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-simulating-lymphadenitis-on-18f-fdg-pet-ct-and-its-differential-diagnosis
#14
Jing Lu, Qingquan Fang, Chao Ma, Fu Su, Guoqiang Chen, Meiling Huang, Weixing Wang, Xinhui Su
Diagnosis of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is challenging as its clinical presentation is atypical. Here we present a case of atypical EBV-HLH simulating lymphadenitis on fluorrine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT), with a view to consider this kind of EBV-HLH as a possible differential diagnosis in lymphadenitis. A 68 years old male who had episodic fever accompanied by weight loss and weakness for two weeks was studied...
November 27, 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29175175/the-relationship-between-circulating-concentration-of-amh-and-lh-content-in-the-follicle-stimulating-hormone-fsh-preparations-on-follicular-growth-and-ovulatory-response-to-superovulation-in-water-buffaloes
#15
A K Redhead, N Siew, N Lambie, D Carnarvon, R Ramgattie, M Knights
The relationship between circulating concentration of anti-mullerian hormone (AMH) and the LH content of follicle stimulating hormone (FSH) preparation on follicular growth and ovulatory response in water buffaloes was evaluated. A single blood sample was taken from cows (N=31; age: 9.06±0.98years) to determine systemic AMH. Animals with concentrations higher or lower than 194±30pg/ml were placed into LOW and HIGH AMH groups and were assigned randomly to be superovulated FSH containing either a high (FSHp, HLH) or low (FolltropinV, LLH) LH content...
November 10, 2017: Animal Reproduction Science
https://www.readbyqxmd.com/read/29170398/arabidopsis-rss1-mediates-cross-talk-between-glucose-and-light-signaling-during-hypocotyl-elongation-growth
#16
Manjul Singh, Aditi Gupta, Dhriti Singh, Jitendra P Khurana, Ashverya Laxmi
Plants possess exuberant plasticity that facilitates its ability to adapt and survive under challenging environmental conditions. The developmental plasticity largely depends upon cellular elongation which is governed by a complex network of environmental and phytohormonal signals. Here, we report role of glucose (Glc) and Glc-regulated factors in controlling elongation growth and shade response in Arabidopsis. Glc controls shade induced hypocotyl elongation in a dose dependent manner. We have identified a Glc repressed factor REGULATED BY SUGAR AND SHADE1 (RSS1) encoding for an atypical basic helix-loop-helix (bHLH) protein of unknown biological function that is required for normal Glc actions...
November 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29166737/-clinical-analysis-of-patients-with-hemophagocytic-lymphohistiocytosis-complicated-with-gastrointestinal-bleeding
#17
Z L Jin, Y N Wang, Z Wang
Objective: To explore the clinical features of patients with hemophagocytic lymphohistiocytosis (HLH) complicated with gastrointestinal bleeding. Methods: The clinical data of 52 patients with HLH diagnosed from January 2015 to February 2017 were analyzed retrospectively. Results: ①Of the 52 patients[including 36 cases of EBV related (69.2%) , 5 cases of lymphoma related (9.6%) , 3 cases of primary HLH (5.8%) , 3 cases of rheumatism related (5.8%) , 2 cases of tuberculosis related (3.8%) , 3 cases of unknown causes (3...
October 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29166736/-prognosis-of-the-central-nervous-system-involvement-in-patients-with-hemophagocytic-lymphohistiocytosis
#18
F Y Wen, L Xiao, Y Xian, X H Wen, X M Guan, M L Liao, J Yu
Objective: To investigate the characteristics and prognostic factor of central nervous system (CNS) involvement in patients with hemophagocytic lymphohistiocytosis (HLH) . Methods: From January 2006 to October 2015, 152 patients with HLH, 88 patients had CNS involvement, their clinical data were collected, and survival was analyzed using the Kaplan-Meier life table method, univariate and multivariate Cox regression model analyses were applied to identify the risk factors of prognosis. Results: ①57.9% patients complicated with neurological symptoms, cerebrospinal fluid abnormalities were observed in 37...
October 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29162540/genomic-analysis-of-nf-%C3%AE%C2%BAb-signaling-pathway-reveals-its-complexity-in-crassostrea-gigas
#19
Mingjia Yu, Jianming Chen, Yongbo Bao, Jun Li
NF-κB signaling pathway is an evolutionarily conserved pathway that plays highly important roles in several developmental, cellular and immune response processes. With the recent release of the draft Pacific oyster (Crassostra gigas) genome sequence, we have sought to identify the various components of the NF-κB signaling pathway in these mollusks and investigate their gene structure. We further constructed phylogenetic trees to establish the evolutionary relationship of the oyster proteins with their homologues in vertebrates and invertebrates using BLASTX and neighbor-joining method...
November 18, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29157204/characterization-of-a-novel-splicing-mutation-in-unc13d-gene-through-amplicon-sequencing-a-case-report-on-hlh
#20
Dongling Liu, Xijiang Hu, Xiwen Jiang, Bo Gao, Cheng Wan, Changying Chen
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disease caused by uncontrolled proliferation of activated lymphocytes and macrophages. Six genes including SH2D1A, PRF1, UNC13D, STX11, STXBP2 and XIAP were reported as causative genes in most cases. CASE PRESENTATION: Here we report a novel splicing mutation in UNC13D gene, which was identified in an 18-year-old female. Patient was diagnosed as HLH base on HLH-2004 guidelines, no history of inherited diseases was revealed in this family, parents were healthy and non-consanguineous...
November 21, 2017: BMC Medical Genetics
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