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haemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28255145/infection-associated-haemophagocytic-syndrome-in-severe-dengue-infection-a-case-series-in-a-district-hospital
#1
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
#2
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
https://www.readbyqxmd.com/read/28222654/is-coronary-arteritis-a-feature-in-secondary-haemophagocytic-lymphohistiocytosis
#3
Beng Teong See, Ke Xin Yip, Hak Lee Ang
Haemophagocytic lymphohistiocytosis (HLH) is rare. Although Kawasaki disease (KD) has been reported as a precursor to HLH, coronary arteritis occurring at the onset of secondary HLH, not in association with KD, has not been reported. An 8-year-old girl presented with virus-induced secondary HLH associated with a giant aneurysm and ectasia of the coronary arteries which was detected incidentally at onset of the disease. She did not fulfill the criteria for diagnosis of KD. The coronary lesions improved after 6 months of treatment with dexamethasone and etoposide...
February 22, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28214404/malignancy-associated-haemophagocytic-lymphohistiocytosis-in-adults
#4
Naval Daver, Hagop Kantarjian
No abstract text is available yet for this article.
February 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28169428/hax1-mutation-positive-children-presenting-with-haemophagocytic-lymphohistiocytosis
#5
Tuba H Karapınar, Deniz Yılmaz Karapinar, Yeşim Oymak, Yılmaz Ay, Bengü Demirağ, Ayça Aykut, Hüseyin Onay, Filiz Hazan, Yeşim Aydınok, Ferda Özkınay, Canan Vergin
The genetic basis of haemophagocytic lymphohistiocytosis (HLH) has not been elucidated in 10% of affected patients. In this study, we report four HLH episodes in three patients with HAX1 gene mutations. We screened the mutations associated with congenital neutropenia (CN) because the neutropenia persisted following HLH treatment. There were homozygous HAX1 mutations detected in all patients. This is the first case series of patients with CN caused by HAX1 mutation who presented with HLH. We hypothesize that severe neutropenia persists after an HLH episode in children without HLH mutations (especially infants) because these patients have CN caused by HAX1 mutations...
February 7, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28095869/macrophage-activation-syndrome-different-mechanisms-leading-to-a-one-clinical-syndrome
#6
REVIEW
Claudia Bracaglia, Giusi Prencipe, Fabrizio De Benedetti
BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAIN CONTENT: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH)...
January 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28093696/secondary-haemophagocytic-lymphohistiocytosis-triggered-by-postnatally-acquired-cytomegalovirus-infection-in-a-late-preterm-infant
#7
Christine Silwedel, Eric Frieauff, Wolfgang Thomas, Johannes G Liese, Christian P Speer
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with impairment of cytotoxic T-cells and natural killer cells. Causes in infants are mostly hereditary immune defects as well as various infectious triggering factors, amongst these cytomegalovirus (CMV). Vertical CMV transmission may occur in utero, during birth, and by breast feeding. Usually, a CMV infection transmitted via breast milk is symptomatic only in very immature preterm infants. We report on a late preterm infant born after 35 + 5 weeks of gestation with a birth weight of 1840 g, being admitted to our intensive care unit at the age of 9 weeks with acute enteritis and severe dehydration...
January 16, 2017: Infection
https://www.readbyqxmd.com/read/28072947/what-s-beneath-the-surface-stevens-johnson-syndrome-toxic-epidermal-necrolysis-combined-with-haemophagocytic-lymphohistiocytosis-a-case-report
#8
U M Nic Ionmhain, B R Knezevic, A Barraclough, M Lucas, M Anstey
No abstract text is available yet for this article.
January 2017: Anaesthesia and Intensive Care
https://www.readbyqxmd.com/read/28057010/update-on-lysinuric-protein-intolerance-a-multi-faceted-disease-retrospective-cohort-analysis-from-birth-to-adulthood
#9
Wladimir Mauhin, Florence Habarou, Stéphanie Gobin, Aude Servais, Anaïs Brassier, Coraline Grisel, Célina Roda, Graziella Pinto, Despina Moshous, Fahd Ghalim, Pauline Krug, Nelly Deltour, Clément Pontoizeau, Sandrine Dubois, Murielle Assoun, Louise Galmiche, Jean-Paul Bonnefont, Chris Ottolenghi, Jacques de Blic, Jean-Baptiste Arnoux, Pascale de Lonlay
BACKGROUND: Lysinuric protein intolerance (LPI) is a rare metabolic disease resulting from recessive-inherited mutations in the SLC7A7 gene encoding the cationic amino-acids transporter subunit y(+)LAT1. The disease is characterised by protein-rich food intolerance with secondary urea cycle disorder, but symptoms are heterogeneous ranging from infiltrative lung disease, kidney failure to auto-immune complications. This retrospective study of all cases treated at Necker Hospital (Paris, France) since 1977 describes LPI in both children and adults in order to improve therapeutic management...
January 5, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27941111/combining-an-antiviral-with-rituximab-in-ebv-related-haemophagocytic-lymphohistiocytosis-led-to-rapid-viral-clearance-and-a-comprehensive-review
#10
Christos Stefanou, Christiana Tzortzi, Fotini Georgiou, Chrystalla Timiliotou
Epstein-Barr virus (EBV)-related haemophagocytic lymphohistiocytosis (EBVr-HLH) has a better prognosis when the virus is rapidly cleared, but the best antiviral approach is controversial. We present a patient to whom the therapeutic standard rituximab was co-administered with valacyclovir and an HLH-specific treatment with favourable viral and clinical responses. We conducted an extensive literature review and contacted several world reference centres and experts to inquire about their approaches and experience...
December 9, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27938384/macrophage-activation-syndrome-triggered-by-coeliac-disease-a-unique-case-report
#11
J Palman, J May, C Pilkington
BACKGROUND: Macrophage activation syndrome is described as a "clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response" in the context of an autoinflammatory or rheumatic disease. Current associations of macrophage activation syndrome with autoimmune disease most notably include a host of rheumatological conditions and inflammatory bowel disease. Epidemiological studies have shown that macrophage activation syndrome is precipitated by autoimmune disease more commonly than previously thought...
December 9, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27842758/characterisation-of-successive-acinetobacter-baumannii-isolates-from-a-deceased-haemophagocytic-lymphohistiocytosis-patient
#12
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...
January 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/27831908/diagnosing-haemophagocytic-syndrome
#13
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...
March 2017: 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
#14
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/27790499/a-case-of-macrophage-activation-syndrome-with-acute-respiratory-distress-syndrome
#15
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
#16
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
#17
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
#18
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/27697804/haemophagocytic-lymphohistiocytosis-an-elusive-syndrome
#19
REVIEW
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
#20
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
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