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syndrome hemophagocytic

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https://www.readbyqxmd.com/read/29152265/hemophagocytic-syndrome-in-pregnancy-case-report-diagnosis-treatment-and-prognosis
#1
Aline Rousselin, Zarrin Alavi, Emmanuelle Le Moigne, Sarah Renard, Christophe Tremouilhac, Aurélien Delluc, Philippe Merviel
Diagnosis of hemophagocytic syndrome remains a challenge in particular during pregnancy. Concomitant presence of clinical and biological signs, for example, fever, pancytopenia, hyperferritinemia, and hypertriglyceridemia, should alert clinicians to suspect HPS and proceed to prompt treatments.
November 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29138019/a-patient-with-severe-fever-with-thrombocytopenia-syndrome-and-hemophagocytic-lymphohistiocytosis-associated-involvement-of-the-central-nervous-system
#2
Kaneko Masahiko, Shikata Hisaharu, Matsukage Shoichi, Maruta Masaki, Shinomiya Hiroto, Suzuki Tadaki, Hasegawa Hideki, Shimojima Masayuki, Saijo Masayuki
Severe fever with thrombocytopenia syndrome (SFTS), a severe infectious disease caused by novel bunyavirus, SFTS virus (SFTSV), is endemic to China, Korea, and Japan. Most SFTS patients show abnormalities in consciousness. Pathological findings in the central nervous system (CNS) of SFTS patients are not reported. A 53-year-old Japanese man was admitted to Uwajima City Hospital with an 8-day history of fever and diarrhea. Laboratory tests revealed leukopenia, thrombocytopenia, and liver enzyme elevation. He was diagnosed as having severe fever with thrombocytopenia syndrome (SFTS) following detection of the SFTSV genome in his blood...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29130133/infusion-of-leukocytes-from-hla-haplo-identical-familial-donors-as-an-adjuvant-in-the-hlh-2004-protocol-to-treat-the-virus-associated-adult-hemophagocytic-lymphohistiocytosis-a-retrospective-study-of-26-patients
#3
Hui Zhang, Zhiming Dai, Nan Yang, Jin Wang, Aili He, Jianli Wang, Yang Zhang, Shan Meng, Baiyan Wang, Rong Sun, Wanggang Zhang
Adult hemophagocytic lymphohistiocytosis (HLH) is a fatal disease with poor survival and a limited role of drug therapies. To help to recognize virus and enhance survival, we infused leukocytes derived from human leukocyte antigen (HLA) haplo-identical familial donors to patients. We retrospectively investigated 26 adult virus-associated hemophagocytic syndrome (VAHS) patients' medical records from 2006-2017. Eleven of the 26 patients accepted relatives' derived leukocytes infusions in addition to drug therapies recommended in the HLH-2004 protocol...
November 12, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29099692/macrophage-activation-syndrome-associated-with-systemic-lupus-erythematosus-treated-successfully-with-the-combination-of-steroid-pulse-immunoglobulin-and-tacrolimus
#4
Natsuki Aoyama-Maeda, Taro Horino, Osamu Ichii, Yoshio Terada
Macrophage activation syndrome (MAS), a variant of secondary hemophagocytic lymphohistiocytosis, is a potentially life-threatening complication of inflammatory and autoimmune diseases. We present a case of MAS as a rare manifestation of systemic lupus erythematosus. Although initial treatment with corticosteroid, with or without cyclosporine A, is justified in patients with MAS, evidence regarding the effectiveness of this treatment protocol remains to be clarified. Our patient was successfully treated with a combination of intravenous immunoglobulin therapy and intravenous methyl predonisolone pulse therapy, which was followed by a course of oral prednisolone and oral tacrolimus...
November 1, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/29077164/macrophage-activation-syndrome-as-a-complication-of-juvenile-rheumatoid-arthritis
#5
Q An, M-W Jin, X-J An, S-M Xu, L Wang
OBJECTIVE: Juvenile rheumatoid arthritis (JRA), also known as juvenile idiopathic arthritis (JIA), is a rare autoimmune joint disorder of children. The concrete causes for the prevalence of the above pathological state are still unknown. In other words, it is an arthritis affecting mainly children and adolescents. Clinically, it has 3 different clinical subtypes. JRA patients are often noticed with some confirmed symptoms including coagulopathy, disseminated intravascular coagulation (DIC) with hepatosplenomegaly, fall in erythrocyte sedimentation rate and higher levels of liver enzymes leading to a life-threatening outcome...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29064197/-reactive-hemophagocytic-syndrome
#6
Fiona Novotny, Federico Simonetta, Kaveh Samii, Yves Chalandon, Jacques Serratrice
The reactive hemophagocytic syndrome comes from an overstimulation of the immune system which causes a cytokine storm. This is a life-threatening condition caracterised by a febrile cytopenia, hepatosplenomegaly and multi-organ failure. The diagnosis is not easy and the HScore can be useful, looking at hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia. The evidence of hemophagocytosis in the bone marrow is not necessary nor sufficient to make the diagnosis but is part of the workup. The underlying cause has to be actively sought, typically an infectious, malignant or autoimmune disorder...
October 18, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29025045/clinical-features-and-outcomes-in-secondary-adult-hemophagocytic-lymphohistiocytosis
#7
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
#8
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
#9
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...
December 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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28910610/platelet-transfusion-refractoriness-in-patients-with-acute-myeloid-leukemia-treated-by-intensive-chemotherapy
#18
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
#19
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
#20
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...
October 2017: Journal of Clinical Immunology
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