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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/29025045/clinical-features-and-outcomes-in-secondary-adult-hemophagocytic-lymphohistiocytosis
#1
Meng Zhou, Li Li, Qiaolei Zhang, Shanshan Ma, Jianai Sun, Lixia Zhu, Danlei Lu, Jingjing Zhu, De Zhou, Yanlong Zheng, Xiudi Yang, Mixue Xie, Mingyu Zhu, Xiujin Ye, Wanzhuo Xie
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease, with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality and challenging diagnosis. METHODS: To improve the recognition and understanding of this disease, we analyzed clinical and laboratory findings and prognostic factors from 205 adult patients diagnosed with HLH in a large cohort managed at a single medical center from January 2011 to December 2015...
September 15, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28970704/hemophagocytic-lymphohistiocytosis-masquerading-as-acute-liver-failure-a-single-center-experience
#2
Nitin Jagtap, Mithun Sharma, Gupta Rajesh, Padaki Nagaraja Rao, Sekaran Anuradha, Manu Tandan, Mohan Ramchandani, Duvvuru Nageshwar Reddy
BACKGROUND/AIM: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH. METHODS: Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study...
September 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28957822/comparing-hemophagocytic-lymphohistiocytosis-in-pediatric-and-adult-patients
#3
Christian A Wysocki
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) has long been thought of primarily as a pediatric disease. However, this syndrome may occur secondary to underlying malignancies, infections, and autoimmune diseases, in adult patients. Here, we seek to highlight similarities and differences between pediatric and adult HLH, knowledge gaps, and areas of active research. RECENT FINDINGS: Malignancy is a more frequent driver of HLH in adults, present in nearly half...
September 27, 2017: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28944149/a-case-of-leprosy-erythema-nodosum-leprosum-and-hemophagocytic-syndrome-a-continuum-of-manifestations-of-same-agent-host-interactions
#4
Prasan Kumar Panda, Ramjas Prajapati, Arvind Kumar, Manisha Jana, Pradeep Immanuel, Pranay Tanwar, Naveet Wig
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28938698/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#5
REVIEW
Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou, Fei Li
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly...
August 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28938257/childhood-visceral-leishmaniasis-distinctive-features-and-diagnosis-of-a-re-emerging-disease-an-11-year-experience-from-a-tertiary-referral-center-in-athens-greece
#6
Panagiotis Krepis, Adamantia Krepi, Ioanna Argyri, Anastasios Aggelis, Alexandra Soldatou, Maria Tsolia
BACKGROUND: Visceral leishmaniasis (VL) remains a public health issue in Greece. The aim of this study was to describe the clinical and epidemiological characteristics of pediatric VL in our region as well as to evaluate the laboratory findings and the diagnostic techniques that are applied. METHODS: We retrospectively reviewed the medical records of all children diagnosed with VL in an 11-year period at a tertiary public hospital in the region of Athens. Demographic features, clinical information and laboratory findings were accessed...
September 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28936212/how-viruses-contribute-to-the-pathogenesis-of-hemophagocytic-lymphohistiocytosis
#7
Ellen Brisse, Carine H Wouters, Graciela Andrei, Patrick Matthys
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primary, genetic HLH as in the secondary, acquired form. Infections with herpesviruses such as Epstein-Barr virus and cytomegalovirus are the most common...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28935695/confirmed-efficacy-of-etoposide-and-dexamethasone-in-hlh-treatment-long-term-results-of-the-cooperative-hlh-2004-study
#8
Elisabet Bergsten, AnnaCarin Horne, Maurizio Aricó, Itziar Astigarraga, R Maarten Egeler, Alexandra H Filipovich, Eiichi Ishii, Gritta Janka, Stephan Ladisch, Kai Lehmberg, Kenneth L McClain, Milen Minkov, Scott Montgomery, Vasanta Nanduri, Diego Rosso, Jan-Inge Henter
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the HLH-94 study, with an estimated 5-year probability of survival (pSu) of 54% (95% CI, 48-60%), systemic therapy included etoposide, dexamethasone and, from week nine, cyclosporine A (CSA). HSCT was indicated in patients with familial/genetic, relapsing, or severe and persistent disease. In HLH-2004, CSA was instead administered upfront, aiming to reduce pre-HSCT mortality and morbidity...
September 21, 2017: Blood
https://www.readbyqxmd.com/read/28932715/hemophagocytic-lymphohistiocytosis-hlh-elusive-diagnosis-of-disseminated-mycobacterium-avium-complex-infection
#9
Eloy E Ordaya, Sulieman Abu Jarir, Robert Yoo, Pranatharthi H Chandrasekar
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of hyperinflammation leading to an uncontrolled and ineffective immune response, associated with high mortality. CASE REPORT: A 26-year-old woman with acute lymphoblastic leukemia, 8 months in remission, was found to have HLH. Without any improvement, stem cell transplantation was considered. Then, Mycobacterium avium complex (MAC) infection was identified as cause of her HLH. With appropriate therapy for infection, HLH improved and transplantation was averted...
September 2017: Germs
https://www.readbyqxmd.com/read/28918754/macrophage-activation-like-syndrome-an-immunological-entity-associated-with-rapid-progression-to-death-in-sepsis
#10
Evdoxia Kyriazopoulou, Konstantinos Leventogiannis, Anna Norrby-Teglund, Georgios Dimopoulos, Aikaterini Pantazi, Stylianos E Orfanos, Nikoletta Rovina, Iraklis Tsangaris, Theologia Gkavogianni, Elektra Botsa, Eleftheria Chassiou, Anastasia Kotanidou, Christina Kontouli, Panagiotis Chaloulis, Dimitrios Velissaris, Athina Savva, Jonas-Sundén Cullberg, Karolina Akinosoglou, Charalambos Gogos, Apostolos Armaganidis, Evangelos J Giamarellos-Bourboulis
BACKGROUND: A subanalysis of a randomized clinical trial indicated sepsis survival benefit from interleukin (IL)-1 blockade in patients with features of the macrophage activation-like syndrome (MALS). This study aimed to investigate the frequency of MALS and to develop a biomarker of diagnosis and prognosis. METHODS: Patients with infections and systemic inflammatory response syndrome were assigned to one test cohort (n = 3417) and a validation cohort (n = 1704)...
September 18, 2017: BMC Medicine
https://www.readbyqxmd.com/read/28914368/macrophage-activation-syndrome-in-still-s-disease-analysis-of-clinical-characteristics-and-survival-in-paediatric-and-adult-patients
#11
Piero Ruscitti, Carmela Rago, Luciana Breda, Paola Cipriani, Vasiliki Liakouli, Onorina Berardicurti, Francesco Carubbi, Caterina Di Battista, Alberto Verrotti, Roberto Giacomelli
Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, complicating Still's disease, both in paediatric and adult patients. In this work, we aimed to investigate clinical picture and outcome of Still's disease patients developing MAS. We performed a retrospective analysis of patients, both paediatrics and adults, affected by Still's disease attending our department. During the follow-up, each patient was investigated for MAS occurrence and possible predictors, clinical and laboratory factors, were analysed...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28910610/platelet-transfusion-refractoriness-in-patients-with-acute-myeloid-leukemia-treated-by-intensive-chemotherapy
#12
Thibault Comont, Suzanne Tavitian, Laurent Bardiaux, Marylise Fort, Bénédicte Debiol, Danièle Morère, Emilie Bérard, Eric Delabesse, Isabelle Luquet, Salima Martinez, Françoise Huguet, Christian Récher, Sarah Bertoli
Platelet transfusion refractoriness (PTR) is a major adverse event in the management of acute myeloid leukemia (AML). In a series of 897 adult patients with AML receiving intensive chemotherapy, we identified 41 patients (4.8%) with PTR. PTR was more frequently observed in parous women, patients with extra-medullary disease, a low white blood cell count, an infection, or hemophagocytic syndrome. Among the 31 patients with anti-human leucocyte antigen (HLA) antibodies, an HLA-matched donor was identified for 18 patients (58...
October 2017: Leukemia Research
https://www.readbyqxmd.com/read/28878816/a-child-with-anorexia-nervosa-presenting-with-severe-infection-with-cytopenia-and-hemophagocytosis-a-case-report
#13
Masao Suda, Shinichiro Nagamitsu, Masahiro Kinosita, Michiko Matsuoka, Shuichi Ozono, Yasushi Otsu, Yushiro Yamashita, Toyojiro Matsuishi
BACKGROUND: Patients with anorexia nervosa in the acute phase have physical complications, such as infectious disease. Although hemophagocytic syndrome due to infection is a rare complication in anorexia nervosa, early identification for hemophagocytosis is important for avoiding a life-threatening condition. CASE PRESENTATION: We report a case of a 12-year-old girl with anorexia nervosa presenting with infection with cytopenia and hemophagocytosis during initial nutritional therapy...
2017: BioPsychoSocial Medicine
https://www.readbyqxmd.com/read/28871523/a-personalized-diagnostic-and-treatment-approach-for-macrophage-activation-syndrome-and-secondary-hemophagocytic-lymphohistiocytosis-in-adults
#14
Bharat Kumar, Sohaib Aleem, Hana Saleh, Jennifer Petts, Zuhair K Ballas
OBJECTIVE: We assessed the clinical features and outcomes based on therapeutic options adopted during hospital stay for adult patients with macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis (MAS/sHLH). METHODS: We conducted a retrospective chart review of all adult patients (age ≥ 18 years) diagnosed with MAS/sHLH at our center between 2010 and 2015. Inclusion criteria for patients were diagnosis of MAS/sHLH during admission and patients meeting at least 5 out of 8 of Henter's criteria or at least 4 out of 6 of the criteria that were tested...
September 4, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28869447/high-concentration-of-mir-133-is-a-useful-marker-for-the-diagnosis-of-lymphoma-associated-hemophagocytic-syndrome
#15
Wuping Li, Yun Zhong, Yuerong Shuang, Hui Huang, Yan Huang, Li Yu, Xianbao Huang
BACKGROUND: Lymphoma associated hemophagocytic syndrome (LAHS) is one of the major adult secondary hemophagocytic lymphohistiocytosis (HLH). Early diagnosis and treatment contribute to improved outcome. No enlarge lymph nodes can often delay the diagnosis of underlying lymphoma. OBJECTIVE: To find out criteria distinguishing LAHS from HLH induced by benign diseases. METHODS: clinical characteristic and laboratory feature of 31 patients with HLH (10 benign disease-associated HLH and 21 LAHS) were analyzed retrospectively...
August 23, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28866302/hemophagocytic-syndrome-primary-forms-and-predisposing-conditions
#16
REVIEW
Fernando E Sepulveda, Geneviève de Saint Basile
Hemophagocytic lymphohistiocytosis (HLH, also referred to a hemophagocytic syndrome) is a life-threatening condition in which uncontrolled activation of lymphocytes and macrophages, and thus the secretion of large amounts of inflammatory cytokines, leads to a severe hyperinflammatory state. Over the last few decades, researchers have characterized primary forms of HLH caused by genetic defects that impair lymphocytes' cytotoxic machinery. Other genetic causes of HLH not related to impaired cytotoxicity have also recently been identified...
August 31, 2017: Current Opinion in Immunology
https://www.readbyqxmd.com/read/28864373/significance-of-hyperferritinemia-in-hospitalized-adults
#17
Meredith Schaffner, Lori Rosenstein, Zuhair Ballas, Manish Suneja
BACKGROUND: Although high ferritin levels are associated with iron overload, it is known that ferritin is also an acute-phase reactant that may be elevated in conditions associated with acute and chronic inflammation. In addition, an elevated ferritin level is a criterion for the diagnosis of hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Therefore, the significance of elevated serum ferritin is often unclear. As HLH/MAS is a medical emergency, prompt diagnosis is important to guide appropriate treatment...
August 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28863861/neuromyelitis-optica-atypical-hemophagocytic-lymphohistiocytosis-and-heterozygous-perforin-a91v-mutation
#18
Boaz Palterer, Francesca Brugnolo, Elena Sieni, Alessandro Barilaro, Paola Parronchi
Neuromyelitis optica is an autoimmune demyelinating inflammatory disease characterized by optic neuritis and myelitis with anti-aquaporin 4 antibodies. Hemophagocytic lymphohistiocytosis is a severe systemic inflammatory syndrome that can present in a genetic primary form or secondarily to infective, neoplastic or autoimmune diseases. Our case discusses the first reported case of atypical late-onset hemophagocytic lymphohistiocytosis in a patient with neuromyelitis optica, with multiple triggering factors and carrying the common A91V hypomorphic perforin mutation, that blurs the distinction between primary and secondary forms...
October 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28860338/hif1a-is-a-critical-downstream-mediator-for-hemophagocytic-lymphohistiocytosis
#19
Rui Huang, Yoshihiro Hayashi, Xiaomei Yan, Jiachen Bu, Jieyu Wang, Yue Zhang, Yile Zhou, Yuting Tang, Lingyun Wu, Zefeng Xu, Xin Liu, Qianfei Wang, Jianfeng Zhou, Zhijian Xiao, James P Bridges, Rebecca A Marsh, Kejian Zhang, Michael B Jordan, Yuhua Li, Gang Huang
Hemophagocytic lymphohistiocytosis is a life-threatening syndrome characterized by overwhelming immune activation. A steroid and chemotherapy-based regimen remains as the first-line of therapy but it has substantial morbidity. Thus, novel, less toxic therapy for hemophagocytic lymphohistiocytosis is urgently needed. Although differences exist between familial hemophagocytic lymphohistiocytosis and secondary hemophagocytic lymphohistiocytosis, they have many common features. Using bioinformatic analysis with familial hemophagocytic lymphohistiocytosis and systemic juvenile idiopathic arthritis, which is associated with secondary hemophagocytic lymphohistiocytosis, we identified a common hypoxia-inducible factor 1A (HIF1A) signature...
August 31, 2017: Haematologica
https://www.readbyqxmd.com/read/28838727/primary-hemophagocytic-lymphohistiocytosis-and-macrophage-activation-syndrome-the-importance-of-timely-clinical-differentiation
#20
EDITORIAL
Alexei A Grom
No abstract text is available yet for this article.
October 2017: Journal of Pediatrics
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