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haemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28752571/haematopoietic-stem-cell-transplantation-for-primary-immunodeficiency-syndromes-a-5-year-single-centre-experience
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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...
May 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28095869/macrophage-activation-syndrome-different-mechanisms-leading-to-a-one-clinical-syndrome
#17
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
#18
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...
June 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
#19
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
#20
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
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