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haemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/29115762/interdisciplinary-care-of-a-neonate-with-haemophagocytic-lymphohistiocytosis
#1
Naomi Lowe-Lennon, Tracey Jones
Haemophagocytic lymphohistiocytosis (HLH) is a rare condition not commonly observed in neonatal units. It poses a challenge to neonatal staff to source expertise and information when diagnosing and treating patients with this condition. This article uses a case study of a neonate with HLH to analyse interdisciplinary team dynamics in hospital and explore how teams can effectively share knowledge and learn from each other when treating patients with rare and complex conditions.
November 7, 2017: Nursing Children and Young People
https://www.readbyqxmd.com/read/29024023/chronic-active-epstein-barr-virus-infection-with-cutaneous-lymphoproliferation-haemophagocytosis-in-the-skin-and-haemophagocytic-syndrome
#2
S Tokoro, T Namiki, K Miura, K Watanabe, A Arai, K Imadome, H Yokozeki
Patients with chronic active Epstein-Barr virus (EBV) infections (CAEBV) present with cutaneous manifestations including hydroa vacciniforme-like eruptions.(1) Haemophagocytic lymphohistiocytosis (HLH), a fatal complication, can occur in severe cases of CAEBV.(2,3) We retrieved 25 cases of CAEBV treated in our hospital and histopathologically characterized the skin lesions of 3 of those cases, including one case with haemophagocytosis. This is a novel report of CAEBV with haemophagocytosis in the skin. This article is protected by copyright...
October 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28956548/epstein-barr-virus-associated-haemophagocytic-lymphohistiocytosis-presenting-with-acute-sensorineural-hearing-loss-a-case-report-and-review-of-the-literature
#3
Ferhat Arslan, Ergenekon Karagöz, Hüseyin Saffet Beköz, Bahadir Ceylan, Ali Mert
Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016)...
September 1, 2017: Le Infezioni in Medicina
https://www.readbyqxmd.com/read/28925994/chimeric-antigen-receptor-t-cell-therapy-assessment-and-management-of-toxicities
#4
REVIEW
Sattva S Neelapu, Sudhakar Tummala, Partow Kebriaei, William Wierda, Cristina Gutierrez, Frederick L Locke, Krishna V Komanduri, Yi Lin, Nitin Jain, Naval Daver, Jason Westin, Alison M Gulbis, Monica E Loghin, John F de Groot, Sherry Adkins, Suzanne E Davis, Katayoun Rezvani, Patrick Hwu, Elizabeth J Shpall
Immunotherapy using T cells genetically engineered to express a chimeric antigen receptor (CAR) is rapidly emerging as a promising new treatment for haematological and non-haematological malignancies. CAR-T-cell therapy can induce rapid and durable clinical responses, but is associated with unique acute toxicities, which can be severe or even fatal. Cytokine-release syndrome (CRS), the most commonly observed toxicity, can range in severity from low-grade constitutional symptoms to a high-grade syndrome associated with life-threatening multiorgan dysfunction; rarely, severe CRS can evolve into fulminant haemophagocytic lymphohistiocytosis (HLH)...
September 19, 2017: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/28911364/tb-iris-presenting-with-haemophagocytic-lymphohistiocytosis-in-a-non-hiv-infected-male
#5
Yu-Chao Tseng, Hsin-Yun Sun, Jui-Hung Tsai, Po-Pin Hung, Jann-Tay Wang
No abstract text is available yet for this article.
October 1, 2017: International Journal of Tuberculosis and Lung Disease
https://www.readbyqxmd.com/read/28852831/uterine-intravascular-lymphoma-as-a-cause-of-fever-of-unknown-origin
#6
REVIEW
Jérôme Hadjadj, Hubert Nielly, Eve Piekarski, Wendy Cuccuini, Bénedicte Deau-Fischer, Muriel Hourseau, Khadija Benali, Claire Fieschi, Marc Aletti, Thomas Papo, Eric Oksenhendler, Lionel Galicier, David Boutboul
Primary intravascular large B cell lymphoma (IVL) remains a diagnostic challenge because of non-specific clinical, laboratory and imaging findings. The aim of the study was to analyse the major characteristics of IVL with uterine involvement. We retrospectively collected features of IVL with uterine involvement that was proven histologically or demonstrated by significant (18)FDG uptake on (18)FDG-PET/CT. Findings were compared to a comprehensive literature review. Five patients were identified. All of them were admitted for fever of unknown origin (FUO), with haemophagocytic lymphohistiocytosis in three cases...
November 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28826312/spontaneous-resolution-of-epstein-barr-virus-induced-haemophagocytic-lymphohistiocytosis-in-still-s-disease-treated-by-tocilizumab-a-case-report
#7
E Ebstein, H-K Ea
No abstract text is available yet for this article.
August 21, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28752571/haematopoietic-stem-cell-transplantation-for-primary-immunodeficiency-syndromes-a-5-year-single-centre-experience
#8
Melissa Norman, Clementine David, Brynn Wainstein, John B Ziegler, Richard Cohn, Richard Mitchell, Tracey O'Brien, Susan Russell, Toby Trahair, Annette Trickett, Katie Frith, Paul Gray
AIM: Haematopoietic stem cell transplantation (HSCT) is a central therapy in the treatment of primary immunodeficiency diseases (PIDs). Over the past 5 years, outcomes have been greatly improved due to earlier diagnosis, improved donor availability, advancements in graft manipulation and the use of less toxic preparative regimens. We present a 5-year audit of HSCT for PID at a single Australian tertiary hospital. METHODS: Retrospective case note review identified diagnosis, pre-transplant medical morbidity, transplant protocol, engraftment, adverse events, post-transplant immune reconstitution and general health...
July 28, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28689492/autoimmune-haemolytic-anaemia-and-haemophagocytic-lymphohistiocytosis-in-an-adolescent-boy-with-tuberculosis-an-unusual-association
#9
Anirban Mandal, Kana Ram Jat, Ashok Singh, Asit Ranjan Mridha, Sushil K Kabra
No abstract text is available yet for this article.
July 2017: Tropical Doctor
https://www.readbyqxmd.com/read/28589450/central-nervous-system-involvement-in-adults-with-haemophagocytic-lymphohistiocytosis-a-single-center-study
#10
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...
August 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28571052/melioidosis-with-possible-haemophagocytic-lymphohistiocytosis
#11
Junaid Beig, Kerry Read, Darren Welch, Hasan Bhally
No abstract text is available yet for this article.
June 2, 2017: New Zealand Medical Journal
https://www.readbyqxmd.com/read/28529117/new-markers-for-adult-onset-still-s-disease
#12
Stéphane Mitrovic, Bruno Fautrel
Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis≥10,000 cells/mm(3) with neutrophils≥80%)...
May 18, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28512105/double-hit-lymphoma-presenting-as-haemophagocytic-lymphohistiocytosis
#13
Xavier Issac Rivera, Lee J McGhan, Jonathan H Schatz, Soham D Puvvada
No abstract text is available yet for this article.
May 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28468603/fluorescence-in-situ-hybridization-is-superior-for-monitoring-epstein-barr-viral-load-in-infectious-mononucleosis-patients
#14
Pengfei Cao, Meili Zhang, Wei Wang, Yafei Dai, Buqing Sai, Jun Sun, Lujuan Wang, Fan Wang, Guiyuan Li, Juanjuan Xiang
BACKGROUND: Epstein Barr virus (EBV) plays a causal role in some diseases, including infectious mononucleosis, lymphoproliferative diseases and nasopharyngeal carcinoma. Detection of EBV infection has been shown to be a useful tool for diagnosing EBV-related diseases. In the present study, we compared the performance of molecular tests, including fluorescence in situ hybridization (FISH) and EBV real-time PCR, to those of serological assays for the detection of EBV infection. METHODS: Thirty-eight patients with infectious mononucleosis (IM) were enrolled, of whom 31 were diagnosed with a mild type, and seven were diagnosed with IM with haemophagocytic lymphohistiocytosis and chronic active EBV infection...
May 3, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28446488/eculizumab-in-a-child-with-atypical-haemolytic-uraemic-syndrome-and-haemophagocytic-lymphohistiocytosis-triggered-by-cytomegalovirus-infection
#15
Gloria M Fraga-Rodriguez, Sonia Brió-Sanagustin, Eulalia Turón-Viñas, Bradley P Dixon, Eduardo Carreras-González
We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration.Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28446487/haemophagocytic-lymphohistiocytosis-as-a-consequence-of-untreated-b-cell-chronic-lymphocytic-leukaemia
#16
Chris Bailey, Claire Dearden, Kirit Ardeshna
Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome characterised by defective cytotoxic function and hypercytokinaemia leading to macrophage expansion and haemophagocytosis. Patients often present with unexplained fevers, hepatosplenomegaly and pancytopenia, with elevation in serum ferritin and triglyceride. Acquired forms are triggered by infection, malignancy or rheumatological disorders. HLH in the setting of chronic lymphocytic leukaemia is rarely reported, however, and is usually associated with infection or as a consequence of chemotherapy...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28433984/haemophagocytic-lymphohistiocytosis-presenting-as-hellp-syndrome-a-diagnostic-and-therapeutic-challenge
#17
Robert Noel Kerley, Raymond Michael Kelly, Mary Rose Cahill, Louise Clare Kenny
Haemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, haematological disorder, which can be clinically challenging to diagnose and manage. We report a case of HLH in a previously healthy 33-year-old primigravida. The patient presented at 22 weeks gestation with dyspnoea, abdominal pain, anaemia, thrombocytopenia and elevated liver enzymes suggestive of HELLP syndrome.HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets is considered a severe form of pre-eclampsia...
April 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28356254/haemophagocytic-lymphohistiocytosis-associated-with-fulminant-hepatitis-and-multiorgan-failure-following-primary-epstein-barr-virus-and-herpes-simplex-virus-type-1-infection
#18
Claudia Honsig, Sandra Beinhardt, Josef Tomasits, Hans Peter Dienes
We present a case of severe fatal hepatitis in a young patient presumably triggered by two ubiquitous viral diseases which occurred in close succession. This case is unusual because of the exceptional chronological sequence of primary Epstein-Barr virus and herpes simplex virus type 1 infection causing systemic immune dysregulation associated with rapidly developing liver failure and consecutive multiorgan failure. Clinical, laboratory and histopathological findings indicated the development of secondary haemophagocytic lymphohistiocytosis triggered by these closely succeeding viral primary infections...
March 29, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28255145/infection-associated-haemophagocytic-syndrome-in-severe-dengue-infection-a-case-series-in-a-district-hospital
#19
Y S Y Jasmine, S L Lee, F K Kan
Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disorder resulting from uncontrolled hyperinflammatory response. There had been increase in cases of one of the secondary form of HLH, i.e., infectionassociated haemophagocytic syndrome (IAHS) in severe dengue in recent years. However, the condition remains under diagnosed due to lack of awareness compounded by the lack of validated diagnostic criteria. Severe hepatitis with prolonged cytopenias, severe hyperferritinemia, hypofibrinogenemia and persistent fever were evident in all four cases reported...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28228435/crohn-s-disease-complicated-by-epstein-barr-virus-driven-haemophagocytic-lymphohistiocytosis-successfully-treated-with-rituximab
#20
Grace Thompson, Dominic Pepperell, Ian Lawrence, Benjamin David McGettigan
We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication...
February 22, 2017: BMJ Case Reports
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