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https://www.readbyqxmd.com/read/27842758/characterisation-of-successive-acinetobacter-baumannii-isolates-from-a-deceased-haemophagocytic-lymphohistiocytosis-patient
#1
Hyeon Jin Choi, Min Cheol Kil, Ji-Young Choi, Sun Ju Kim, Ki-Sup Park, Yae-Jean Kim, Kwan Soo Ko
In this study, 38 Acinetobacter baumannii isolates successively isolated from blood, skin swabs and tracheal aspirates from a single patient who died from haemophagocytic lymphohistiocytosis were investigated. The isolates were collected between March 2012 and August 2012. A. baumannii genotypes were determined by multilocus sequence typing (MLST) and pulsed-field gel electrophoresis (PFGE). In vitro antimicrobial susceptibility testing was performed and colistin heteroresistance and persistence were evaluated...
November 8, 2016: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/27831908/diagnosing-haemophagocytic-syndrome
#2
REVIEW
Ethan S Sen, Colin G Steward, Athimalaipet V Ramanan
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment...
October 24, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27793796/diffuse-18f-fluorodeoxyglucose-accumulation-in-the-bone-marrow-of-a-patient-with-haemophagocytic-lymphohistiocytosis-due-to-hodgkin-lymphoma
#3
Shu Harada, Tsutomu Shinohara, Keishi Naruse, Hisanori Machida
No abstract text is available yet for this article.
October 28, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27793107/acute-hiv-infection-presenting-as-hemophagocytic-syndrome-with-an-unusual-serological-and-virological-response-to-art
#4
Rita Veiga Ferraz, Ana Cláudia Carvalho, Fernando Araújo, Carmo Koch, Cândida Abreu, António Sarmento
BACKGROUND: HIV clinical presentation in the acute stage is variable and some of its virological and immunological aspects are not completely understood. Most cases of HIV- associated reactive hemophagocytic syndrome have been reported in patients with advanced stages of HIV and to our knowledge, there are only 8 cases in the English literature presenting during acute HIV infection, most in East Asia, being this the first case in a European patient. CASE PRESENTATION: We report a case of a European Caucasian 27- year old woman with a primary HIV- infection presenting with extremely low CD4+ T cell count who developed a haemophagocytic syndrome after starting ART and in whom we documented a very unusual serological and virological response, characterized by an impaired HIV- antibody production and a 12 month time frame to reach an undetectable viral load, despite no evidence of resistance...
October 28, 2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27790499/a-case-of-macrophage-activation-syndrome-with-acute-respiratory-distress-syndrome
#5
Robin George Manappallil
Macrophage Activation Syndrome (MAS) is a rare form of Haemophagocytic Lymphohistiocytosis (HLH) associated with rheumatologic conditions. It is characterised by haemophagocytosis and cytokine overproduction, resulting from the activation and uncontrolled proliferation of T lymphocytes and macrophages. The patient being reported is an elderly female with Rheumatoid Arthritis (RA), who presented with fever and was found to have MAS. She later developed Acute Respiratory Distress Syndrome (ARDS) and died. Such a scenario has rarely been reported...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27773211/the-importance-of-repeating-a-bone-marrow-examination-a-fatal-case-of-epstein-barr-virus-ebv-driven-haemophagocytic-lymphohistiocytosis-hlh-in-a-1-year-old
#6
Rebecca Barton, Anthea Greenway, Janine Campbell
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27772828/haemophagocytic-lymphohistiocytosis
#7
Joanne Brown, Meena Shingde, Winny Varikatt
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27738405/salmonella-glomerulonephritis-and-haemophagocytic-lymphohistiocytosis-in-an-adolescent
#8
Seçil Arslansoyu Çamlar, Mustafa Kır, Ceyda Aydoğan, Şebnem Yılmaz Bengoa, Mehmet Atilla Türkmen, Alper Soylu, Salih Kavukçu
No abstract text is available yet for this article.
September 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/27722764/subcutaneous-panniculitis-like-t-cell-lymphoma-immunosuppressive-drugs-induce-better-response-than-polychemotherapy
#9
David Michonneau, Tony Petrella, Nicolas Ortonne, Saskia Ingen-Housz-Oro, Nathalie Franck, Stéphane Barete, Maxime Battistella, Marie Beylot-Barry, Béatrice Vergier, Marc Maynadié, Christine Bodemer, Olivier Hermine, Martine Bagot, Nicole Brousse, Sylvie Fraitag
Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare condition usually considered as favourable. However, it is not known whether polychemotherapy or immunosuppressive-based therapy is the best approach for treating SPTCL. Using data collected between 2000 and 2012 in France, we analysed clinical, biological and pathological data of 27 patients with SPTCL. Medical history revealed that 40% of patients had been previously diagnosed with an autoimmune disorder and 22% with inflammatory panniculitis...
October 10, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27697804/haemophagocytic-lymphohistiocytosis-an-elusive-syndrome
#10
William Thomas, Mars Van't Veer, Martin Besser
Haemophagocytic lymphohistiocytosis is a rare inflammatory condition. It can present in adult general medical patients and is a challenging diagnostic conundrum. This article provides an overview of the pathophysiology and clinical presentation of the syndrome for the general physician who will be rarely confronted with this problem but will have to act promptly when the situation arises. Treatment is also briefly discussed, although this usually occurs in a specialist setting after the diagnosis has been established...
October 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/27622035/missense-mutations-in-the-perforin-prf1-gene-as-a-cause-of-hereditary-cancer-predisposition
#11
Mohammed S Chaudhry, Kimberly C Gilmour, Imran G House, Mark Layton, Nicki Panoskaltsis, Mamta Sohal, Joseph A Trapani, Ilia Voskoboinik
Perforin, a pore-forming toxin released from secretory granules of NK cells and CTLs, is essential for their cytotoxic activity against infected or cancerous target cells. Bi-allelic loss-of-function mutations in the perforin gene are invariably associated with a fatal immunoregulatory disorder, familial haemophagocytic lymphohistiocytosis type 2 (FHL2), in infants. More recently, it has also been recognized that partial loss of perforin function can cause disease in later life, including delayed onset FHL2 and haematological malignancies...
July 2016: Oncoimmunology
https://www.readbyqxmd.com/read/27609213/plasmodium-falciparum-and-mycoplasma-pneumoniae-co-infection-presenting-with-cerebral-malaria-manifesting-orofacial-dyskinesia-and-haemophagocytic-lymphohistiocytosis
#12
Praveen Weeratunga, Gowri Rathnayake, Ahalya Sivashangar, Panduka Karunanayake, Ariaranee Gnanathasan, Thashi Chang
BACKGROUND: Malaria is a mosquito-borne infectious disease with diverse clinical manifestations caused by a parasitic protozoan of the genus Plasmodium. Complex inter-relationships between Mycoplasma species and Plasmodium parasites have been previously noted in vitro. This is the first report of Plasmodium falciparum and Mycoplasma pneumoniae co-infection in a human host presenting with cerebral malaria manifesting orofacial dyskinesias and haemophagocytic lymphohistiocytosis. CASE PRESENTATION: A 55-year-old Sri Lankan man with a recent visit to South Africa presented with an acute febrile illness, cough and worsening dyspnoea with alveolar-interstitial infiltrates on chest radiography...
2016: Malaria Journal
https://www.readbyqxmd.com/read/27576518/brain-damage-resembling-acute-necrotizing-encephalopathy-as-a-specific-manifestation-of-haemophagocytic-lymphohistiocytosis-induced-by-hypersensitivity
#13
Dongling Dai, Feiqiu Wen, Sixi Liu, Shaoming Zhou
BACKGROUND: Both haemophagocytic lymphohistiocytosis and acute necrotizing encephalopathy are life-threatening condition. It presents major diagnostic difficulties, since it may have a diversity in clinical picture and with many conditions leading to the same clinical presentation. So it is key important to understand the disorders. CASE PRESENTATION: We report a pediatric case of haemophagocytic lymphohistiocytosis with specific presentation which predominantly featured as acute necrotizing encephalopathy of childhood...
2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27576345/primary-immunodeficiencies-in-chile-evaluated-through-icd-10-coded-hospital-admissions
#14
C Poli, R Hoyos-Bachiloglu, A Borzutzky
BACKGROUND: The epidemiology and hospitalisation trends of primary immunodeficiency (PID) in Chile are unknown. We aimed to evaluate hospitalisation trends and demographic characteristics of PID admissions in Chile. METHODS: PID admissions between 2001 and 2010 (ICD-10 codes D70.0, D70.4, D71, 72.0, D76.1, D80-D84, E70.3, G11.3) were reviewed using national hospital discharge databases. RESULTS: During the study period, 5486 admissions due to PID were registered (0...
August 28, 2016: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/27434021/haemophagocytic-lymphohistiocytosis-and-silvery-hair-in-griscelli-syndrome
#15
Dan Lipsker
No abstract text is available yet for this article.
October 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27408381/a-case-presenting-with-splenic-infarct-diagnosed-as-primary-bone-marrow-cd5-positive-dlbcl-a-clinicopathological-correlation
#16
Anupriya Bansal, Suchi Mittal, Jasmita Dass, Nitin Gupta, P K Agarwal, Jyoti Kotwal
De novo CD5+ Diffuse large B cell lymphoma (DLBCL) is a rare and aggressive subtype of DLBCL. It is a distinct clinicopathologic entity with complex molecular profile and poor prognosis. A 59 year old female presented with pyrexia of unknown origin since 1 month. On examination, there was severe pallor, hepatosplenomegaly and no palpable lymphadenopathy. Complete blood count revealed bicytopenia with normal total leucocyte count. Liver and renal function tests were normal. Ultrasonography abdomen revealed splenic enlargement with two focal lesions attributed to either splenic abscess or infarcts...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27397412/haemophagocytic-lymphohistiocytosis-presenting-as-neonatal-liver-failure-a-case-series
#17
Hala Abdullatif, Nabil Mohsen, Rokaya El-Sayed, Fatma El-Mougy, Hanaa El-Karaksy
BACKGROUND AND STUDY AIM: Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome with liver involvement varying from mild dysfunction to severe fulminant failure. The aim of this study was to present a case series of four HLH patients presenting with acute liver failure (ALF) in the neonatal period. PATIENTS AND METHODS: All four patients were neonates at the onset of symptoms. They presented to Cairo University Pediatric Hospital with ALF; they underwent prompt investigations including determination of ferritin, fibrinogen, and triglyceride levels as part of our ALF workup...
June 2016: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/27387105/the-role-of-18-f-fdg-pet-ct-in-the-management-of-patients-with-secondary-haemophagocytic-lymphohistiocytosis
#18
Y Zheng, G Hu, Y Liu, Y Ma, Y Dang, F Li, H Xing, T Wang, L Huo
AIM: To investigate the ability of combined 2-[(18)F]-fluoro-2-deoxy-d-glucose ((18)F-FDG) positron-emission tomography (PET)/computed tomography (CT) to determine potential causes of secondary haemophagocytic lymphohistiocytosis (sHLH) and to predict prognosis. MATERIAL AND METHODS: Forty-three patients (male/female 20/23, median age 48.5 years), who were diagnosed with sHLH and underwent FDG-PET/CT before treatment, were retrospectively reviewed. The clinical characteristics were compared to identify the predictors of high-yield FDG-PET/CT...
July 4, 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27383696/development-of-spondyloarthropathy-following-episodes-of-macrophage-activation-syndrome-in-children-with-heterozygous-mutations-in-haemophagocytic-lymphohistiocytosis-associated-genes
#19
Randy Q Cron, W Winn Chatham
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July 4, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27367321/an-analysis-of-children-with-brucellosis-associated-with-haemophagocytic-lymphohistiocytosis
#20
Kamuran Karaman, Sinan Akbayram, Sultan Kaba, Serap Karaman, Mesut Garipardiç, Ilyas Aydin, Ahmet Fayik Öner
This retrospective study included seven paediatric cases aged from 4 to 14 (10.2±3.4) years with pathologically proved haemophagocytic lymphohistiocytosis from a single institution during 2009 and 2013. Over this time period, 496 patients with brucellosis were diagnosed. None of the patients (3 boys and 4 girls) had a history of any haematologic disorder. All patients had an anamnesis for recently consumed unpasteurised homemade dairy products or had a contact history with sheep and/or cows. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titres were 1: 1280 in seven patients...
June 1, 2016: Le Infezioni in Medicina
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