keyword
MENU ▼
Read by QxMD icon Read
search

HLH

keyword
https://www.readbyqxmd.com/read/28332776/interferon-gamma-and-interleukin-10-polymorphisms-in-chinese-children-with-hemophagocytic-lymphohistiocytosis
#1
Qi An, Shao-Yan Hu, Cheng-Min Xuan, Ming-Wei Jin, Qiang Ji, Yi Wang
AIM: The aim of the study is to investigate the association of interferon gamma (IFN-γ) and interleukin-10 (IL-10) gene single nucleotide polymorphisms with the susceptibility of hemophagocytic lymphohistiocytosis (HLH) in Chinese children without known family history of HLH. PROCEDURE: Forty children with HLH and 160 age- and gender-matched healthy controls from Xuzhou Children's Hospital were enrolled in the study. Serum IFN-γ and IL-10 levels were measured by enzyme linked-immunosorbent assay...
March 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28328614/a-newborn-with-familial-hemophagocytic-lymphohistiocytosis-complicated-with-transfusion-associated-graft-versus-host-disease
#2
Ahmet Ozdemir, Tamer Gunes, Samuel C C Chiang, Ekrem Unal
Hemophagocytic lymphohistiocytosis (HLH) is characterized by activation of cytotoxic T and natural killer (NK) cells, and macrophages related to a spectrum of hyperinflammatory disorders. The clinical findings mainly include high fever, cytopenia, splenomegaly, phagocytosis, and proliferation of histiocytes in lymphoreticular tissue. To the best of our knowledge, transfusion-associated graft versus host disease (TA-GVHD) in a 13-day old male newborn with HLH is being reported first time in the literature. The aim of this report was to emphasize the importance of blood products irradiation in the prevention of the development of graft versus host disease especially among high-risk subjects such as newborns with HLH...
March 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28286628/hemophagocytic-lymphohistiocytosis-an-unclear-nosologic-entity-case-report-of-an-adult-man-with-rising-of-amylase-and-lipase-and-spinal-cord-infiltration
#3
Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, Carlo Sabbà
Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28283651/tfe3-regulates-whole-body-energy-metabolism-in-cooperation-with-tfeb
#4
Nunzia Pastore, Anna Vainshtein, Tiemo J Klisch, Andrea Armani, Tuong Huynh, Niculin J Herz, Elena V Polishchuk, Marco Sandri, Andrea Ballabio
TFE3 and TFEB are members of the MiT family of HLH-leucine zipper transcription factors. Recent studies demonstrated that they bind overlapping sets of promoters and are post-transcriptionally regulated through a similar mechanism. However, while Tcfeb knockout (KO) mice die during early embryonic development, no apparent phenotype was reported in Tfe3 KO mice. Thus raising the need to characterize the physiological role of TFE3 and elucidate its relationship with TFEB TFE3 deficiency resulted in altered mitochondrial morphology and function both in vitro and in vivo due to compromised mitochondrial dynamics...
March 10, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28270454/perforin-and-cd107a-testing-are-superior-to-nk-cell-function-testing-for-screening-patients-for-genetic-hlh
#5
Tamar S Rubin, Kejian Zhang, Carrie Gifford, Adam Lane, Jack J Bleesing, Rebecca A Marsh
Primary HLH (hemophagocytic lymphohistiocytosis) can be caused by bi-allelic mutations in PRF1, encoding perforin, or UNC13D, STXBP2, STX11, RAB27A, LYST and AP3B1, encoding proteins involved in cytotoxic lymphocyte degranulation. Natural killer (NK) cell cytotoxicity assays can quickly screen for all these genetic diseases, facilitating treatment, but combining NK cell perforin expression and CD107a upregulation tests can as well. To determine the relative diagnostic accuracies for each approach, we retrospectively reviewed screening test performance in 1614 patients referred for HLH evaluation...
March 7, 2017: Blood
https://www.readbyqxmd.com/read/28255145/infection-associated-haemophagocytic-syndrome-in-severe-dengue-infection-a-case-series-in-a-district-hospital
#6
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/28249810/dynamic-changes-of-laboratory-parameters-and-peripheral-blood-lymphocyte-subsets-in-severe-fever-with-thrombocytopenia-syndrome-patients
#7
Jingwen Liu, Li Wang, Zhaolei Feng, Daying Geng, Ye Sun, Guangying Yuan
OBJECTIVES: The aim of this study is to dynamically investigate laboratory parameters and peripheral blood lymphocyte subsets in severe fever with thrombocytopenia syndrome(SFTS)patients at different stages, to evaluate the significance of these changes in the infection process and its influence on prognosis. METHODS: Case-control study was used in the research. Sixty-nine confirmed thrombocytopenia syndrome virus(SFTSV)infected patients were enrolled. They were divided into two groups, recovery group and poor prognosis group, according to the clinical prognosis of the diseases...
February 26, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28238659/the-cdk8-complex-and-proneural-proteins-together-drive-neurogenesis-from-a-mesodermal-lineage
#8
Shuo Luo, H Robert Horvitz
At least some animal species can generate neurons from mesoderm or endoderm, but the underlying mechanisms remain unknown. We screened for C. elegans mutants in which the presumptive mesoderm-derived I4 neuron adopts a muscle-like cell fate. From this screen, we identified HLH-3, the C. elegans homolog of a mammalian proneural protein (Ascl1) used for in vitro neuronal reprogramming, as required for efficient I4 neurogenesis. We discovered that the CDK-8 Mediator kinase module acts together with a second proneural protein, HLH-2, and in parallel to HLH-3 to promote I4 neurogenesis...
March 6, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28228435/crohn-s-disease-complicated-by-epstein-barr-virus-driven-haemophagocytic-lymphohistiocytosis-successfully-treated-with-rituximab
#9
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/28225498/dengue-associated-hemophagocytic-lymphohistiocytosis-in-an-adult-a-case-report-and-literature-review
#10
REVIEW
Sang Mi Chung, Joon Young Song, Wonshik Kim, Min Joo Choi, Ji Ho Jeon, Seonghui Kang, Eunju Jung, Ji Yun Noh, Hee Jin Cheong, Woo Joo Kim
BACKGROUND: Infection-associated hemophagocytic syndrome (IAHS) is potentially a fatal disease caused by systemic infection complicated by hemophagocyticlymphohistiocytosis (HLH). Here, we report a case of HLH associated with dengue hemorrhagic fever (DHF) after a trip to Thailand. CASE SUMMARY: A 33-year-old healthy female patient presented with 3 days of fever, myalgia, and skin rash. Serotype 3 dengue virus was isolated. Clinical and laboratory findings fulfilled the criteria of HLH...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28222654/is-coronary-arteritis-a-feature-in-secondary-haemophagocytic-lymphohistiocytosis
#11
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/28222313/cytokine-profiles-as-novel-diagnostic-markers-of-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-children
#12
Xiu-Cui Han, Qing Ye, Wei-Ying Zhang, Yong-Min Tang, Xiao-Jun Xu, Ting Zhang
PURPOSE: The aim of this study was to identify specific laboratory indices to distinguish Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children. MATERIALS AND METHODS: In this prospective study, Th1/Th2 cytokines, including IL-2, IL-4, IL-6, IL-10, TNF-α, and IFN-γ, were analyzed in patients with EBV-HLH or sepsis at the onset of disease by flow cytometry. RESULTS: IL-10, IFN-γ, IL-10/IL-6, and IFN-γ/IL-6 were higher and IL-6 was lower in EBV-HLH patients compared to sepsis patient levels...
February 10, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28219834/incidence-and-outcomes-of-central-nervous-system-hemophagocytic-lymphohistiocytosis-relapse-after-reduced-intensity-conditioning-hematopoietic-stem-cell-transplantation
#13
Dana T Lounder, Pooja Khandelwal, Sharat Chandra, Michael B Jordan, Ashish R Kumar, Michael Grimley, Stella M Davies, Jack J Bleesing, Rebecca A Marsh
Hemophagocytic lymphohistiocytosis (HLH) is an immune regulatory disorder that commonly presents with central nervous system (CNS) involvement. The only cure for genetic HLH is hematopoietic stem cell transplantation (HSCT), typically treated with reduced-intensity conditioning (RIC) regimens. We sought to estimate the incidence of CNS relapse after RIC HSCT, determine risk factors, and evaluate outcomes. We performed a retrospective chart review of 94 consecutive children and young adults with primary HLH who received RIC HSCT...
February 17, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28219570/living-donor-lung-transplantation-after-bone-marrow-transplantation-for-chediak-higashi-syndrome
#14
Tetsu Yamada, Toyofumi F Chen-Yoshikawa, Shigeharu Oh, Rieko Ito-Taniguchi, Fumiaki Gochi, Masaaki Sato, Akihiro Aoyama, Hiroshi Date
An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28210636/hemophagocytic-lymphohistiocytosis-in-a-patient-with-hodgkin-lymphoma-and-concurrent-ebv-cmv-and-candida-infections
#15
Moaath Mustafa Ali, Ana Lucia Ruano Mendez, Hetty E Carraway
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by immune activation and subsequent widespread organ damage. Patients affected by HLH commonly develop fever, cytopenias, liver damage, neurologic manifestations, and hypercytokinemia. In this case, we describe a 60-year-old male who presented with HLH and concurrent Epstein-Barr virus, cytomegalovirus, and Candida infections and was subsequently diagnosed with a Hodgkin lymphoma. This case highlights the importance of considering a cancer diagnosis in the differential diagnosis of patients presenting with HLH...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28202022/hematologic-manifestations-of-babesiosis
#16
REVIEW
Tamer Akel, Neville Mobarakai
BACKGROUND: Babesiosis, a zoonotic parasitic infection transmitted by the Ixodes tick, has become an emerging health problem in humans that is attracting attention worldwide. Most cases of human babesiosis are reported in the United States and Europe. The disease is caused by the protozoa of the genus Babesia, which invade human erythrocytes and lyse them causing a febrile hemolytic anemia. The infection is usually asymptomatic or self-limited in the immunocompetent host, or follows a persistent, relapsing, and/or life threatening course with multi-organ failure, mainly in the splenectomized or immunosuppressed patients...
February 15, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28196537/characterization-of-a-novel-disease-causing-mutation-in-exon-1-of-sh2d1a-gene-through-amplicon-sequencing-a-case-report-on-hlh
#17
Shiyuan Zhou, Hongyu Ma, Bo Gao, Guangming Fang, Yi Zeng, Qing Zhang, GaoFu Qi
BACKGROUND: Hemophagocytic lymphohistocytosis (HLH) is a rare but fatal hyperinflammatory syndrome caused by uncontrolled proliferation of activated macrophages and T lymphocytes secreting high amounts of inflammatory cytokines. Genetic defect is a common cause of HLH. HLH is complicated to be diagnosed as there are many common symptoms with other disorders. CASE PRESENTATION: Here we report on an HLH case caused by 1 bp deletion in gene SH2D1A. Patient was a 3-years-old boy and had fever for more than 8 days...
February 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28193515/infliximab-not-associated-with-increased-risk-of-malignancy-or-hemophagocytic-lymphohistiocytosis-in-pediatric-patients-with-inflammatory-bowel-disease
#18
Jeffrey S Hyams, Marla C Dubinsky, Robert N Baldassano, Richard B Colletti, Salvatore Cucchiara, Johanna Escher, William Faubion, John Fell, Benjamin D Gold, Anne Griffiths, Sibylle Koletzko, Subra Kugathasan, James Markowitz, Frank M Ruemmele, Gigi Veereman, Harland Winter, Nicholas Masel, Chu Ri Shin, Kezhen L Tang, Meena Thayu
BACKGROUND AND AIMS: Immunosuppressive therapy for inflammatory bowel disease (IBD) in pediatric patients is thought to increase risk of malignancy and lymphoproliferative disorders, including hemophagocytic lymphohistiocytosis (HLH). We compared unadjusted incidence rates and of malignancy and HLH in pediatric patients with IBD exposed to infliximab compared with patients not exposed to biologics and calculated standardized incidence ratios (SIRs). METHODS: We collected and analyzed data from 5766 participants in a prospective study of long-term outcomes of pediatric patients with IBD (NCT00606346), from 2007 through 30 June 2016...
February 10, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28191297/leishmania-infantum-and-epstein-barr-virus-co-infection-in-a-patient-with-hemophagocytosis
#19
Zied Gaifer, Mohamed-Rachid Boulassel
The authors describe a rare case of a 27- year old previously healthy male presenting with high grade fever, pancytopenia, hepatosplenomegaly, high levels of ferritin and triglyceride, suggesting a diagnosis of hemophagocytic lymphohistiocytosis (HLH) syndrome. Other investigations showed a positive Leishmania infantum serology and high Epstein-Barr virus (EBV) viremia. The diagnosis of a visceral leishmaniasis was confirmed by bone morrow biopsy, which showed Leishman-Donovan bodies and evidence of HLH. The patient received liposomal amphotericin B and he had a complete resolution of his symptoms and clearance of EBV viremia...
December 31, 2016: Infectious Disease Reports
https://www.readbyqxmd.com/read/28188950/hemophagocytic-lymphohistiocytosis-in-a-neonate-case-report
#20
Pari Zarrini, Ziba Mosayebi, Asghar Ramyar, Hosein Dalili
 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This article will introduce a neonate with HLH in Iran. We report a case of HLH presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life. Typical clinical and laboratory findings were detected in the neonate. HLH was diagnosed according to HLH-2004 guidelines...
January 2017: Acta Medica Iranica
keyword
keyword
23588
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"