نتایج جستجو برای: hlh

تعداد نتایج: 1249  

2015
Yuan Li Xuefeng Xia Jing Zhang Zhiqiang Song Liya Zhou Yaopeng Zhang Yonghui Huang Yanyan Shi Eamonn M.M. Quigley Shigang Ding

Patients with inflammatory bowel disease (IBD) are at risk of developing haemophagocytic lymphohistiocytosis (HLH) because of chronic systemic inflammation as well as exposure to immunosuppressive medications. The two main causes of HLH in IBD patients are infection with cytomegalovirus and Epstein-Barr virus. Patients with Crohn's disease are more susceptible to HLH than those with ulcerative ...

2011
Yukayo Ukeba-Terashita Yoshinori Saita Yoshinori Ito Hirokazu Kanegane Hiroshi Kimura Ichiro Kobayashi

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by high grade fever, heaptosplenomegaly, lymphadenopathy and cytopenia in association with hypercytokinemia and macrophage activation. Epstein-Barr virus (EBV) is a common cause of HLH particularly in Asian countries. Although EBV usually infects B cells and causes infectious mononucleosis, it is detected predominantly in...

Journal: :Blood 2017
Melissa R Hines Kim E Nichols

HLH comprises a heterogeneous group of disorders characterized by the excessive activation of CD8 T cells and macrophages that secrete high levels of proinflammatory cytokines. Inherited or “primary” HLH is caused by the presence of germline mutations that negatively affect the killing activity of cytotoxic lymphocytes (CD8 T and natural killer [NK] cells). In contrast, sporadic or “secondary” ...

2016
Karen Put Ellen Brisse Anneleen Avau Maya Imbrechts Tania Mitera Rik Janssens Paul Proost Francesca Fallarino Carine H. Wouters Patrick Matthys Luis Graca

OBJECTIVES Indoleamine 2,3-dioxygenase-1 (IDO1) is an immune-modulatory enzyme that catalyzes the degradation of tryptophan (Trp) to kynurenine (Kyn) and is strongly induced by interferon (IFN)-γ. We previously reported highly increased levels of IFN-γ and corresponding IDO activity in patients with hemophagocytic lymphohistiocytosis (HLH), a hyper-inflammatory syndrome. On the other hand, IFN-...

2016
SOMANATH PADHI

We read with great interest the first case report of hemophagocytic lymphohistiocytosis (HLH) in association with leprosy by Høyvoll et al. They described a young female with multibacillary leprosy (MBL) and erythema nodosum leprosum (ENL). After 3 weeks of instituting multidrug therapy (MDT) with rifampin, dapsone, clofazimine, and prednisolone, she developed high-grade fever and other systemi...

2014
Jing Li Qian Wang Wenjie Zheng Jie Ma Wei Zhang Wenze Wang Xinping Tian

To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, 1aboratory findings, outcomes, and prognostic factors. The most common cause of...

Journal: :Rheumatology 2015
Karen Put Anneleen Avau Ellen Brisse Tania Mitera Stéphanie Put Paul Proost Brigitte Bader-Meunier René Westhovens Benoit J Van den Eynde Ciriana Orabona Francesca Fallarino Lien De Somer Thomas Tousseyn Pierre Quartier Carine Wouters Patrick Matthys

OBJECTIVES To study the role of IFN-γ in the pathogenesis of systemic JIA (sJIA) and haemophagocytic lymphohistiocytosis (HLH) by searching for an IFN-γ profile, and to assess its relationship with other cytokines. METHODS Patients with inactive (n = 10) and active sJIA (n = 10), HLH [n = 5; of which 3 had sJIA-associated macrophage activation syndrome (MAS)] and healthy controls (n = 16) wer...

Journal: :Gastroenterology 2017
Jeffrey S Hyams Marla C Dubinsky Robert N Baldassano Richard B Colletti Salvatore Cucchiara Johanna Escher William Faubion John Fell Benjamin D Gold Anne Griffiths Sibylle Koletzko Subra Kugathasan James Markowitz Frank M Ruemmele Gigi Veereman Harland Winter Nicholas Masel Chu Ri Shin Kezhen L Tang Meena Thayu

BACKGROUND AND AIMS Immunosuppressive therapy for inflammatory bowel disease (IBD) in pediatric patients is thought to increase the risk of malignancy and lymphoproliferative disorders, including hemophagocytic lymphohistiocytosis (HLH). We compared unadjusted incidence rates of malignancy and HLH in pediatric patients with IBD exposed to infliximab (IFX) with patients not exposed to biologics ...

2013
Thanh K. Quach Han Ting Chou Kun Wang Gaolin Zheng Milledge Casonya M. Johnson

In Caenorhabditis elegans, the six proteins that make up the REF-1 family have been identified as functional homologs of the Hairy/Enhancer of Split (HES) proteins. These transcription factors act in both Notch dependent and Notch-independent pathways to regulate embryonic events during development; however, their post-embryonic functions are not well defined. As a first step toward understandi...

Journal: :The EMBO journal 2012
Ryohei Ishii Kazunobu Isogaya Azusa Seto Daizo Koinuma Yuji Watanabe Fumio Arisaka So-ichi Yaguchi Hiroaki Ikushima Naoshi Dohmae Kohei Miyazono Keiji Miyazawa Ryuichiro Ishitani Osamu Nureki

Helix-loop-helix (HLH) family transcription factors regulate numerous developmental and homeostatic processes. Dominant-negative HLH (dnHLH) proteins lack DNA-binding ability and capture basic HLH (bHLH) transcription factors to inhibit cellular differentiation and enhance cell proliferation and motility, thus participating in patho-physiological processes. We report the first structure of a fr...

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