Publications
| Title | Abstract | Year(sorted descending) Filter | PMID Filter |
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| how genetic testing can lead to targeted management of xiap deficiency-related inflammatory bowel disease. | x-linked lymphoproliferative disease type 2 (xlp-2, omim 300635) is a primary immunodeficiency caused by the loss of x chromosome-linked inhibitor of apoptosis (xiap), the x-linked inhibitor of apoptosis gene at xq25. xlp-2 individuals are susceptible to several specific and potentially fatal infections, such as epstein-barr virus (ebv). children with xiap-related xlp-2 may present with either familial hemophagocytic lymphohistiocytosis, often triggered in response to ebv infection, or with a tr ... | 2017 | 27416006 |
| posttransplant lymphoproliferative disorders in irish renal transplant recipients: insights from a national observational study. | posttransplant lymphoproliferative disorders (ptld) are a common malignancy after renal transplantation with a high incidence of ptld described in the first posttransplant year. we sought to determine incidence and risk determinants of ptld in irish kidney transplant recipients. | 2017 | 27214265 |
| role of human papillomavirus and tumor suppressor genes in oral cancer. | the incidence of oral cancer remains high and is associated with many deaths in both western and asian countries. several risk factors for the development of oral cancer are now well known, including smoking, drinking and consumption of smokeless tobacco products. genetic predisposition to oral cancer has been found in certain cases, but its components are not yet entirely clear. in accordance with the multi-step theory of carcinogenesis, the natural history of oral cancer seems to gradually evo ... | 2017 | 27194871 |
| comprehensive profiling of virus micrornas of epstein-barr virus-associated gastric carcinoma: highlighting the interactions of ebv-bart9 and host tumor cells. | epstein-barr virus (ebv) is suggested to actively utilize its ebv-micrornas (mirnas) to manipulate viral and cellular functions during neoplasia transformation. a systemic profiling of ebv-mirnas expressed in ebv-associated gastric carcinoma (ebva gc) helps understand its epigenetic regulation of carcinogenesis. | 2017 | 27144885 |
| rec8 functions as a tumor suppressor and is epigenetically downregulated in gastric cancer, especially in ebv-positive subtype. | rec8 meiotic recombination protein (rec8) was found to be preferentially methylated in gastric cancer (gc) using promoter methylation array. we aimed to elucidate the epigenetic alteration and biological function of rec8 in gc. rec8 was downregulated in 100% (3/3) of epstein-barr virus (ebv)-positive and 80% (8/10) of ebv-negative gc cell lines by promoter methylation, but the expression could be restored through demethylation treatment. protein expression of rec8 was significantly lower in huma ... | 2017 | 27212034 |
| weathering a cytokine storm: a case of ebv-induced hemophagocytic lymphohistiocytosis. | hemophagocytic lymphohistiocytosis (hlh) is a rare but life-threatening disease caused by excessive immune activation. acquired hlh is seen in adults and is often caused by infection or malignancy. diagnosis is difficult and usually missed as clinical and laboratory findings are nonspecific. moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to hlh. thus, in patients presenting with presumed severe sepsis or septic shock complicated b ... | 2017 | 27200381 |
| monitoring of epstein-barr virus load and killer t cells in patients with juvenile idiopathic arthritis treated with methotrexate or tocilizumab. | methotrexate (mtx) is used for the treatment of polyarticular juvenile idiopathic arthritis (jia), and an anti-interleukin-6 receptor monoclonal antibody (tocilizumab: tcz) is also used and added for the treatment of intractable jia. it has been reported that mtx might induce epstein-barr virus (ebv)-associated lymphoma, but the discussion about the effect of mtx and/or tcz against reactivation of ebv in pediatric patients has been incomplete. | 2017 | 27166661 |
| clinicopathological categorization of epstein-barr virus-positive t/nk-cell lymphoproliferative disease: an analysis of 42 cases with an emphasis on prognostic implications. | epstein-barr virus-positive t/nk-cell lymphoproliferative diseases (ebv-t/nk-lpds) include several overlapping ebv-related conditions with variably aggressive courses. for prognostic categorization, we retrospectively analyzed 42 ebv-t/nk-lpd cases. male (79% [33/42]), young (≤40 years; 83% [35/42]) patients and t-cell lineage (81% [34/42]; cd8/cd4 = 1.8) were predominant. clinicopathologically, three systemic and one cutaneous category were developed: hemophagocytic lymphohistiocytosis (hlh; 26 ... | 2017 | 27157296 |
| epstein-barr virus load in transplant patients: early detection of post-transplant lymphoproliferative disorders. | high levels of circulating ebv load are used as a marker of post-transplant lymphoproliferative disorders (ptld). there is no consensus regarding the threshold level indicative of an increase in peripheral ebv dna. the aim of the study was to clinically validate a developed ebv quantification assay for early ptld detection. transversal study: paired peripheral blood mononuclear cells (pbmc), plasma and oropharyngeal lymphoid tissue (olt) from children undergoing a solid organ transplant with (n= ... | 2017 | 27157146 |
| cutaneous extranodal natural killer/t-cell lymphomas histopathologically mimicking benign inflammatory disease. | few studies have evaluated the histopathological features of cutaneous extranodal nk/t-cell lymphoma (enktl). the authors' study aims to describe cases of cutaneous enktl that histopathologically mimic benign inflammatory dermatosis. the authors searched the database of asan medical center to identify cases of cutaneous enktl that were confirmed by skin biopsy between january 1998 and december 2014. the authors included cases that were initially diagnosed as benign inflammatory disorders on the ... | 2017 | 27149332 |
| ebv-associated peripheral t-cell lymphoma of gastrointestinal tract presented with widespread chronic inflammation: a case report. | we report a case of epstein-barr virus (ebv)-associated t-cell lymphoma of gastrointestinal (gi) tract from a 70-year-old white woman who initially presented with a widespread gi inflammation and gastric obstruction. initial biopsies of the gi tract showed severe chronic inflammation in the esophagus, stomach, and the small intestine. celiac disease and inflammatory bowel disease were ruled out. the patient was treated with partial gastrectomy. histology showed gastric wall thickening with ebv-p ... | 2017 | 27093448 |
| pure lymphoepithelioma-like carcinoma originating from the urinary bladder. | lymphoepithelioma-like carcinoma of the urinary bladder (lelcb) is a rare variant of infiltrating urothelial carcinoma. we report a case of lelcb in a 43-year-old man. ultrasonography and cystoscopy revealed two bladder tumors, one on the left side of the trigone and the other on the right side of the trigone. transurethral resection of the bladder tumors was performed and pathological analysis revealed undifferentiated carcinoma. we therefore performed radical cystectomy and urinary diversion. ... | 2017 | 27099604 |
| neurosurgery and human immunodeficiency virus in the era of combination antiretroviral therapy: a review. | human immunodeficiency virus (hiv) is a global health problem. it renders the central nervous system susceptible to infectious and noninfectious diseases. hiv-positive individuals may present to neurosurgical services with brain lesions of unknown etiology. the differential diagnosis in these cases is broad, including opportunistic infections and malignancies, and investigation should be tailored accordingly. opportunistic infections of the central nervous system can be complicated by hydrocepha ... | 2017 | 27081898 |
| cholecystitis and nephrotic syndrome complicating epstein-barr virus primary infection. | epstein-barr virus (ebv) infection results in a spectrum of clinical manifestations. the host immune response to ebv plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. a 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic ... | 2017 | 27077634 |
| epstein-barr virus and its association with rheumatoid arthritis and oral lichen planus. | pathogenesis of autoimmune diseases (ad) is one of a multifactorial milieu. a genetic predisposition, an immune system failure, hormonal imbalance and environmental factors play important roles. among the many environmental factors, the role of infection is gaining importance in the pathogenesis of various autoimmune disorders; among them, epstein-barr virus (ebv) plays a pivotal role. literature states an association of various ad with ebv namely multiple sclerosis, autoimmune thyroiditis, syst ... | 2017 | 26980953 |
| epstein-barr virus encephalitis with substantia nigra involvement. | infectious mononucleosis due to epstein-barr virus (ebv) is a usually benign systemic viral illness common in children. many studies described nervous system manifestations of infectious mononucleosis with a wide spectrum of neurologic deficits. neurologic complications of ebv are seen in both acute and reactivate infection. herein, we describe a patient diagnosed by acute ebv encephalitis with substantia nigra involvement and excellent clinical recovery. | 2017 | 26962357 |
| iatrogenic immunodeficiency-associated lymphoproliferative disorders in transplant and nontransplant settings. | iatrogenic immunodeficiency-associated lymphoproliferative disorders comprise a group of lymphoid neoplasms that are associated with an immunosuppressed state, either in the posttransplant period, or during the treatment of various autoimmune and rheumatologic disorders by immunomodulatory medications. their morphologies vary widely but are generally classified according to the lymphomas that they most closely resemble. this group is strongly associated with infections by the epstein-barr virus ... | 2017 | 26960627 |
| bart mirnas: an unimaginable force in the development of nasopharyngeal carcinoma. | nasopharyngeal carcinoma (npc) is a head and neck cancer that represents a major health burden in southern china and southeast asia. although the close association of npc with epstein-barr virus (ebv) infection has been demonstrated, its exact role in the pathogenesis of this malignancy is still unclear. the expression of ebv-encoded micrornas, especially bart mirnas, which are encoded from the bamhi-a region of the viral genome, is detected at a high level in npc. mirnas are small noncoding mrn ... | 2017 | 26909566 |
| rampant infections of bone marrow stem cell niches as triggers for spondyloarthropathies and rheumatoid arthritis. | tropheryma whipplei can induce rheumatism mimicking spa or ra, but even more rampant bacterial/viral infections in epiphyseal bones could also contribute to the onset of ra and spa. indeed, as bone marrow stem cell niches are enriched in tregs and myeloid derived suppressor cells, these areas are favourable for the persistence of quiescent viruses and/or dormant bacteria. this review focuses on the possibility that such silent infections of bone marrow stem cell niches might contribute to the pa ... | 2017 | 26886813 |
| patterns of pd-l1 expression and cd8 t cell infiltration in gastric adenocarcinomas and associated immune stroma. | recent data supports a significant role for immune checkpoint inhibitors in the treatment of solid tumours. here, we evaluate gastric and gastro-oesophageal junction (g/gej) adenocarcinomas for their expression of programmed death-ligand 1 (pd-l1), infiltration by cd8+ t cells and the relationship of both factors to patient survival. | 2017 | 26801886 |
| lymphoepithelioma-like carcinoma of the urinary bladder: a rare case report. | bladder cancers are the second most common urogenital malignancy, its most common type being urothelial carcinoma. lymphoepithelioma-like carcinoma (lelc) which is commonly described in the nasopharynx is a very rare presentation in the bladder. diagnosis of this entity poses a histopathological challenge. nonetheless, the correct diagnosis is important as it implies a different therapeutic approach with the potential bladder salvage treatment protocol. we here present a case of an 87-year-old m ... | 2017 | 26692678 |
| a case of mature natural killer-cell neoplasm manifesting multiple choroidal lesions: primary intraocular natural killer-cell lymphoma. | natural killer (nk) cell neoplasm is a rare disease that follows an acute course and has a poor prognosis. it usually emerges from the nose and appears in the ocular tissue as a metastasis. herein, we describe a case of nk-cell neoplasm in which the eye was considered to be the primary organ. | 2017 | 26668579 |
| nhl - extranodal t-cell lymphoma. | extranodal nk/t-cell lymphoma, nasal type (enkl) is a rare lymphoid neoplasm that in the past has been grouped with a variety of granulomatous diseases. enkl occurs in all age groups. however, it seems to occur more often in people in their 50s and affects more men than women. it is strongly linked to the epstein-barr virus (ebv), especially in people of asian countries. because this type of lymphoma occurs in organs or tissues other than lymph nodes, it is called enkl. this is a case report of ... | 2017 | 26668464 |
| molecular concept in human oral cancer. | the incidence of oral cancer remains high in both asian and western countries. several risk factors associated with development of oral cancer are now well-known, including tobacco chewing, smoking, and alcohol consumption. cancerous risk factors may cause many genetic events through chromosomal alteration or mutations in genetic material and lead to progression and development of oral cancer through histological progress, carcinogenesis. oral squamous carcinogenesis is a multistep process in wh ... | 2017 | 26668446 |
| extranodal natural killer/t-cell lymphoma, nasal type: a great pretender. | extranodal natural killer/t-cell lymphoma, nasal type (enktcl) is a rare epstein-barr virus associated lymphoma seen predominantly in asian population with a 5 years survival rate ranging from 10% to 75% depending on the stage of presentation. in this case report, we describe an unusual presentation of enktcl, which in its early stages was clinically misdiagnosed as buccal space infection and later on histologically as inflammatory myofibroblastic pseudotumor owing to manifold reasons. postopera ... | 2017 | 26539376 |
| cd5-positive primary intraocular b-cell lymphoma arising during methotrexate and tumor necrosis factor inhibitor treatment. | to report a case of cd5+ primary intraocular b-cell lymphoma arising during methotrexate (mtx) and tumor necrosis factor (tnf) inhibitor treatment in a young patient with rheumatoid arthritis and uveitis. | 2017 | 26483672 |
| stable persistence of the yeast plasmid by hitchhiking on chromosomes during vegetative and germ-line divisions of host cells. | the chromosome-like stability of the saccharomyces cerevisiae plasmid 2 micron circle likely stems from its ability to tether to chromosomes and segregate by a hitchhiking mechanism. the plasmid partitioning system, responsible for chromosome-coupled segregation, is comprised of 2 plasmid coded proteins rep1 and rep2 and a partitioning locus stb. the evidence for the hitchhiking model for mitotic plasmid segregation, although compelling, is almost entirely circumstantial. direct tests for plasmi ... | 2017 | 26442178 |
| joint involvement secondary to epstein-barr virus. | we describe a group of patients with epstein-barr virus (ebv) infection and joint involvement. between february 2011 and january 2012, there were six cases in our unit. two presented with a pattern similar to rheumatoid arthritis, three had polyarthralgia with an inflammatory pattern and only one patient had asymmetrical oligoarthritis of large joints. they were all women aged between 25 and 75 (4 were of child-bearing potential). diagnosis in all the cases was made by exclusion of other possibl ... | 2017 | 26441062 |
| clinicopathologic characteristics of microsatellite instable gastric carcinomas revisited: urgent need for standardization. | microsatellite instable gastric cancer (msi-gc) is a specific molecular subtype of gc. we studied the phenotypes, genotypes, and clinicopathologic characteristics of msi-gc in a white gc cohort and compared our findings with an extended literature review. the study cohort consisted of 482 patients. specimens were available from 452 cases and were used for immunostaining (mlh1, pms2, msh2, msh6) and molecular biological analyses (bat-25, bat-26, nr-21, nr-24, nr-27; epstein-barr virus in situ hyb ... | 2017 | 26371427 |
| the presence of human papillomavirus and epstein-barr virus in male chinese lichen sclerosus patients: a single center study. | this study aimed to investigate the presence of human papillomavirus and epstein-barr virus in male lichen sclerosus patients. we extracted dna from formalin-fixed paraffin-embedded foreskin tissue blocks of 47 male ls patients and 30 healthy men and performed real-time pcr test to detect hpv and ebv. none of the 47 ls patients and 30 healthy men had detectable hpv infection. ebv was detected in 18 ls patients (38.3%) and four healthy men (13.3%), the difference is significant (p < 0.05). tissue ... | 2017 | 26289401 |
| the prognostic significance of ebv dna load and eber status in diagnostic specimens from diffuse large b-cell lymphoma patients. | epstein-barr virus (ebv)-encoded small rna in situ hybridization (eber-ish) is a widely accepted method to evaluate ebv involvement in diffuse large b-cell lymphoma (dlbcl), although little is known regarding associations between ebv dna load and the eber status and whether ebv dna load data provide additional clinical information. in this study, we quantified ebv dna load in diagnostic specimens from dlbcl patients diagnosed at our hospital to evaluate clinical implications of ebv dna load in d ... | 2017 | 26177728 |
| extranodal nk/t-cell lymphoma mimicking erythema multiforme. | extranodal natural killer/t-cell lymphoma (enktl) is an extremely rare and an aggressive lymphoma. we report an interesting case of extranodal natural killer/t-cell lymphoma in a middle-aged woman which mimicked erythema multiforme. the skin lesions of this case looked like erythema multiforme due to the tagetoid lesions and mucosal erosions. the two biopsies from her chest and scalp lesions were different. one exhibited the characteristics of cd4+ cd56+ cd8-mycosis fungoides whereas the other b ... | 2017 | 26120182 |
| evaluation of the association between epstein-barr virus and mycosis fungoides. | mycosis fungoides (mf) is the most common cutaneous t-cell lymphomas. despite extensive studies, etiopathogenesis of mf is unknown. environmental, infectious and genetic factors have been proposed as potential risk factors of mf. herpes virus family members, especially epstein-barr virus (ebv), have been among the viral factors of interest in recent years. the aim of this study was to investigate the possible association of ebv infection with mf. | 2017 | 26120176 |
| optic neuritis following epstein-barr virus encephalitis in immunocompetent children: a case report. | 2017 | 26073488 | |
| candidate gene association studies and risk of hodgkin lymphoma: a systematic review and meta-analysis. | to evaluate the contribution of association studies of candidate polymorphisms to inherited predisposition to hodgkin lymphoma (hl), we conducted a systematic review and meta-analysis of published case-control studies. of the variants examined more than once in candidate gene association studies, we identified 21 studies that reported on 12 polymorphic variants in 10 genes. data were also extracted from a published genome wide association study to allow analysis of an additional 47 variants in a ... | 2017 | 26053036 |
| ebv-associated lymphoepithelioma-like carcinoma of the pancreas: case report with targeted sequencing analysis. | lymphoepithelioma-like carcinomas are distinctive epithelial derived malignant neoplasms that have a syncytial growth pattern and lymphoid stroma. the majority of tumors with this appearance are epstein barr virus (ebv)-associated. we report a patient with a clinical presentation concerning for lymphoma who was diagnosed with an ebv-associated pancreatic carcinoma with a lymphoepithelioma-like pattern. targeted sequencing analysis showed a molecular profile distinct from conventional ductal aden ... | 2017 | 25922198 |
| toll-like receptor gene polymorphisms and susceptibility to epstein-barr virus-associated and -negative gastric carcinoma in northern china. | various polymorphisms in toll-like receptor (tlr) genes have been identified and associated with susceptibility to various malignancies, such as gastric carcinoma (gc), breast cancer, and prostate cancer. however, little is known about the polymorphisms of tlr genes and the susceptibility to gc in northern china, especially to epstein-barr virus-associated gc (ebvagc). we focused on the association with susceptibility to gc, especially to ebvagc. | 2017 | 25843196 |
| cytokeratin-negative undifferentiated (lymphoepithelial) carcinoma of the lacrimal sac. | epstein-barr virus-associated undifferentiated (lymphoepithelial) carcinoma is a malignancy that most commonly arises in the nasopharynx but can also occur in other locations including the lacrimal sac. generally, this tumor strongly expresses cytokeratin, making the diagnosis straightforward. in the absence of confirmatory immunohistochemistry, the diagnosis can be problematic, particularly for tumors arising in unusual locations. only 3 cases arising in the lacrimal sac in association with eps ... | 2017 | 25719371 |
| angioimmunoblastic t-cell lymphoma: a diagnostic challenge. | angioimmunoblastic t-cell lymphoma (aitl) accounts for 15-20% of all peripheral t-cell lymphomas. it is a rare subtype of cd4 t-cell peripheral lymphoma that affects aged individuals, causing b symptoms, generalized lymphadenopathy and hepatosplenomegaly. its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure. the majority of patients are diagnosed in an advanced stage and anthracycline based regimen is considered the first- ... | 2017 | 25685133 |
| epstein -barr virus association with malignant lymphoma subgroups in zaria, nigeria. | epstein-barr virus (ebv) is said to infect more than 90% of humans worldwide with latent infection for life. a recognized carcinogen, ebv is linked to malignant lymphoma (ml) subtypes of burkitt's lymphoma (bl), plasmablastic lymphoma, diffuse large cell lymphoma (dlbcl) and hodgkin's lymphoma (hl). we report the association of ebv with ml in a segment of our patient population. paraffin blocks from the archives of abuth, zaria were used to construct tissue microarray sections stained using 30 m ... | 2017 | 25620871 |
| [macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis]. | machrophage activation syndrome (mas) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. it is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia ... | 2017 | 25575650 |
| hemophagocytic lymphohistiocytosis secondary to epstein barr virus and leishmania co-infection in a toddler. | this is the report of an ebv+leishmanial co-infection. the patient developed hemophagocytic syndrome (hlh) and was treated with the standard hlh-2004 protocol. however, pcr in bone marrow discovered this secondary cause for hlh. in endemic countries, visceral leishmaniasis should be considered in the differential diagnosis even in ebv-related hlh, as chemotherapy toxicity may be avoided. | 2017 | 25511219 |
| hepatosplenic hodgkin lymphoma without lymphadenopathy following reversible methotrexate-associated lymphoproliferative disorder. | lymphoproliferative disorders (lpds) occur more frequently in rheumatoid arthritis (ra) patients treated with immunosuppressive agents than in the non-ra population. however, the various forms of disease progression have not yet been elucidated in detail. we encountered a case of epstein-barr virus (ebv)-positive atypical polymorphous lpd in the cervical and intraabdominal lymph nodes with hepatosplenomegaly in an 88-year-old female with ra who had taken infliximab and methotrexate (mtx) for six ... | 2017 | 25401225 |
| tracheal epstein-barr virus-associated smooth muscle tumour in an hiv-positive patient. | epstein-barr virus-related smooth muscle tumours (ebv-smts) are a rare but well recognized non-aids-defining malignancy that can also be found in several other immunosuppressed states. pulmonary involvement of ebv-smts is not uncommon, but it can present with multifocal lesions in any anatomical site. the present article describes an hiv-positive woman with dyspnea who was found to have a large tracheal ebv-smt. the authors discuss their approach to diagnosis and management, and present unique f ... | 2017 | 25255459 |
| interferon beta-1a treatment in htlv-1-associated myelopathy/tropical spastic paraparesis: a case report. | here a young patient (< 21 years of age) with a history of infective dermatitis is described. the patient was diagnosed with myelopathy associated with htlv-1/tropical spastic paraparesis and treated with interferon beta-1a. the disease was clinically established as htlv-1-associated myelopathy/tropical spastic paraparesis (ham/tsp), and laboratory tests confirmed the presence of antibodies to htlv-1 in the cerebrospinal fluid (csf). mumps, cytomegalovirus, epstein-barr virus, schistosomiasis, h ... | 2017 | 25229227 |
| intravascular large b-cell lymphoma: a difficult diagnostic challenge. | case presentation. a 69-year-old hispanic male, with a past history of diabetes and coronary disease, was admitted for fever, diarrhea, and confusion of 4 weeks duration. physical examination showed a disoriented patient with multiple ecchymoses, possible ascites, and bilateral scrotal swelling. hemoglobin was 6.7, prothrombin time (pt) 21.4 seconds with international normalized ratio 2.1, partial thromboplastin time (ptt) 55.6 seconds, fibrin split 10 µg/l, and lactate dehydrogenase (ldh) 1231 ... | 2017 | 26425595 |
| molecular mimicry of mage-a6 and mycoplasma penetrans hf-2 epitopes in the induction of antitumor cd8(+) t-cell responses. | a promising vaccine strategy for the treatment of cancer involves the use of vaccines incorporating tumor antigen-derived synthetic peptides that can be coordinately recognized by specific cd4(+) and cd8(+) t-cells. previously, we reported that a mage-a6-derived peptide (mage-a6172-187) and its highly-immunogenic and cross-reactive homolog derived from mycoplasma penetrans hf-2 permease (hf-2216-229) are promiscuously presented by multiple hla-dr alleles to responder cd4(+) t-cells obtained from ... | 2017 | 25960935 |
| role of infectious agents in cutaneous t-cell lymphoma: facts and controversies. | the etiology of cutaneous t-cell lymphoma (ctcl) remains unknown, with potential infectious causes having been explored. this contribution evaluates the evidence suggesting an infectious etiology and pathogenesis of the disease, characterizes the relationships between various specific pathogens and ctcl, and discusses some of the difficulties in establishing a causal link between infectious agents and ctcl carcinogenesis. researchers have evaluated ctcl specimens for evidence of infection with a ... | 2017 | 23806159 |
| the histological and biological spectrum of diffuse large b-cell lymphoma in the world health organization classification. | diffuse large b-cell lymphomas (dlbcls) are aggressive b-cell lymphomas that are clinically, pathologically, and genetically diverse, in part reflecting the functional diversity of the b-cell system. the focus in recent years has been toward incorporation of clinical features, morphology, immunohistochemistry, and ever evolving genetic data into the classification scheme. the 2008 world health organization classification reflects this complexity with the addition of several new entities and vari ... | 2017 | 23006945 |
| effects of epstein-barr virus on the development of dendritic cells derived from cord blood monocytes: an essential role for apoptosis. | epstein-barr virus (ebv) is a ubiquitous human γ-herpes virus, which can adapt and evade host immune defense. dendritic cells (dcs) play a pivotal role in the initiation and maintenance of immune responses. this study investigated the effects of ebv on cord blood monocytes derived dcs (cbdc). | 2017 | 22358351 |
| t-cell lymphoma/sézary syndrome in a liver transplant recipient. | cutaneous t-cell lymphoma is a rare subtype of posttransplantation lymphoproliferative disease, with only 29 cases reported in the literature to date, the majority of which are post-renal transplantation. only a few were reported after pancreas, heart, and bone marrow transplantation. there have been no reports of cutaneous t-cell lymphoma following liver transplantation to date. | 2017 | 22418000 |
| disarming cellular alarm systems-manipulation of stress-induced nkg2d ligands by human herpesviruses. | the coevolution of viruses and their hosts led to the repeated emergence of cellular alert signals and viral strategies to counteract them. the herpesvirus family of viruses displays the most sophisticated repertoire of immune escape mechanisms enabling infected cells to evade immune recognition and thereby maintain infection. the herpesvirus family consists of nine viruses that are capable of infecting humans: herpes simplex virus 1 and 2 (hsv-1, hsv-2), varicella zoster virus (vzv), epstein-ba ... | 2017 | 28443092 |
| association of cognitive function and liability to addiction with childhood herpesvirus infections: a prospective cohort study. | liability to substance use disorder (sud) is largely nonspecific to particular drugs and is related to behavior dysregulation, including reduced cognitive control. recent data suggest that cognitive mechanisms may be influenced by exposure to neurotropic infections, such as human herpesviruses. in this study, serological evidence of exposure to human herpesvirus herpes simplex virus type 1 (hsv-1), cytomegalovirus (cmv), and epstein-barr virus (ebv) as well as toxoplasma gondii was determined in ... | 2017 | 28420448 |
| pcr-based approach for qualitative molecular analysis of six neurotropic pathogens. | in the last few years, polymerase chain reaction analysis is frequently required to improve the detection of pathogen infections in central nervous system as a potential cause of neurological disorders and neuropsychiatric symptoms. the goal of this paper is to set up a fast, cheap and reliable molecular approach for qualitative detection of six neurotropic pathogens. a method based on pcr has been designed and implemented to guarantee the qualitative dna detection of herpes simplex virus types ... | 2017 | 28854791 |
| herpesviruses excretion in saliva of pediatric transplant recipients. | saliva samples could be used for follow-up of herpesviruses infection in the pediatric transplant recipients. | 2017 | 28834054 |
| molecular diagnosis of central nervous system opportunistic infections and mortality in hiv-infected adults in central china. | csf pcr is the standard diagnostic technique used in resource-rich settings to detect pathogens of the cns infection. however, it is not currently used for routine csf testing in china. knowledge of cns opportunistic infections among people living with hiv in china is limited. | 2017 | 28469695 |
| functionality testing of stem cell grafts to predict infectious complications after allogeneic hematopoietic stem cell transplantation. | allogeneic hematopoietic stem cell transplantation (hsct) is a routine clinical procedure performed to treat patients with haematological malignancies, primary immune deficiencies or metabolic disorders. infections during lymphopenia after allogeneic hsct are associated with high mortality and morbidity. typical infectious agents are epstein-barr virus, cytomegalovirus, herpes simplex virus, varicella-zoster virus and fungi. the study aim was to evaluate whether measurement of the responses of a ... | 2017 | 28466551 |
| [varicella gastritis under immunosuppression : case report of a woman after lung transplantation due to granulomatosis with polyangiitis]. | a 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. the gastroscopy showed diffuse ulcerative gastric lesions. tests for varicella zoster virus and epstein-barr virus via polymerase chain reactions (pcr) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. intravenous antiviral therapy with acyclovir was administered resulting in a normalization of all clinical symptoms, esp ... | 2017 | 28405696 |
| epidemiology of multiple herpes viremia in previously immunocompetent patients with septic shock. | systemic reactivations of herpesviruses may occur in intensive care unit (icu) patients, even in those without prior immune deficiency. however, the clinical relevance of these events is uncertain. | 2017 | 28158551 |
| crystal structure of the dna-binding domain of human herpesvirus 6a immediate early protein 2. | immediate early proteins of human herpesvirus 6a (hhv-6a) are expressed at the outset of lytic infection, and thereby regulate viral gene expression. immediate early protein 2 (ie2) of hhv-6a is a trans-activator that drives a variety of promoters. the c-terminal region of hhv-6a ie2 is shared among ie2 homologs in beta-herpesviruses, and is involved in dimerization, dna binding, and transcription factor binding. in this study, the structure of the c-terminal domain (ie2-ctd) was determined by x ... | 2017 | 28794035 |
| structural basis for potent antibody-mediated neutralization of human cytomegalovirus. | human cytomegalovirus (hcmv) is the leading viral cause of birth defects and organ transplant rejection. the hcmv gh/gl/ul128/ul130/ul131a complex (pentamer) is the main target of humoral responses and thus a key vaccine candidate. we report two structures of pentamer bound to human neutralizing antibodies, 8i21 and 9i6, at 3.0 and 5.9 å resolution, respectively. the hcmv gh/gl architecture is similar to that of epstein-barr virus (ebv) except for amino-terminal extensions on both subunits. the ... | 2017 | 28783665 |
| the human cytomegalovirus nuclear egress complex unites multiple functions: recruitment of effectors, nuclear envelope rearrangement, and docking to nuclear capsids. | nuclear replication represents a common hallmark of herpesviruses achieved by a number of sequentially unrolled regulatory processes. a rate-limiting step is provided by nucleo-cytoplasmic capsid export, for which a defined multiregulatory protein complex, namely, the nuclear egress complex (nec), is assembled comprising both viral and cellular components. the nec regulates at least 3 aspects of herpesviral nuclear replication: (1) multimeric recruitment of nec-associated effector proteins, (2) ... | 2017 | 28664574 |
| a temporal proteomic map of epstein-barr virus lytic replication in b cells. | epstein-barr virus (ebv) replication contributes to multiple human diseases, including infectious mononucleosis, nasopharyngeal carcinoma, b cell lymphomas, and oral hairy leukoplakia. we performed systematic quantitative analyses of temporal changes in host and ebv proteins during lytic replication to gain insights into virus-host interactions, using conditional burkitt lymphoma models of type i and ii ebv infection. we quantified profiles of >8,000 cellular and 69 ebv proteins, including >500 ... | 2017 | 28514666 |
| a histological study of fulminant type 1 diabetes mellitus related to human cytomegalovirus reactivation. | fulminant type 1 diabetes mellitus (t1dm) is thought to be partly caused by virus infection. | 2017 | 28398495 |
| the transcription factor hobit identifies human cytotoxic cd4(+) t cells. | the t cell lineage is commonly divided into cd4-expressing helper t cells that polarize immune responses through cytokine secretion and cd8-expressing cytotoxic t cells that eliminate infected target cells by virtue of the release of cytotoxic molecules. recently, a population of cd4(+) t cells that conforms to the phenotype of cytotoxic cd8(+) t cells has received increased recognition. these cytotoxic cd4(+) t cells display constitutive expression of granzyme b and perforin at the protein leve ... | 2017 | 28392788 |
| effects of metal-rich particulate matter exposure on exogenous and endogenous viral sequence methylation in healthy steel-workers. | inhaled particles have been shown to produce systemic changes in dna methylation. global hypomethylation has been associated to viral sequence reactivation, possibly linked to the activation of pro-inflammatory pathways occurring after exposure. this observation provides a rationale to investigate viral sequence (both exogenous and endogenous) methylation in association to metal-rich particulate matter exposure. to verify this hypothesis, we chose the wp promoter of the epstein-barr virus (ebv-w ... | 2017 | 28858759 |
| recreational exposure during algal bloom in carrasco beach, uruguay: a liver failure case report. | in january 2015, a 20-month-old child and her family took part in recreational activities at carrasco and malvín beaches (montevideo, uruguay). an intense harmful algae bloom (hab) was developing along the coast at that time. a few hours after the last recreational exposure episode, the family suffered gastrointestinal symptoms which were self-limited except in the child's case, who was admitted to hospital in uruguay with diarrhea, vomiting, fatigue, and jaundice. the patient had increased seru ... | 2017 | 28858213 |
| tcr-like antibodies mediate complement and antibody-dependent cellular cytotoxicity against epstein-barr virus-transformed b lymphoblastoid cells expressing different hla-a*02 microvariants. | epstein-barr virus (ebv) is a common gammaherpesvirus associated with various human malignancies. antibodies with t cell receptor-like specificities (tcr-like mabs) provide a means to target intracellular tumor- or virus-associated antigens by recognising their processed peptides presented on major histocompatibility complex (mhc) class i (pmhc) complexes. these antibodies are however thought to be relevant only for a single hla allele. here, we show that hla-a*02:01-restricted ebv antigenic pep ... | 2017 | 28855662 |
| epstein-barr virus, cytomegalovirus, and multiple sclerosis susceptibility: a multiethnic study. | to determine whether epstein-barr virus (ebv) or cytomegalovirus (cmv) seropositivity is associated with multiple sclerosis (ms) in blacks and hispanics and to what extent measures of the hygiene hypothesis or breastfeeding could explain these findings. ebv and cmv have been associated with ms risk in whites, and the timing and frequency of both viruses vary by factors implicated in the hygiene hypothesis. | 2017 | 28855411 |
| human genetic determinants of viral diseases. | much progress has been made in the identification of specific human gene variants that contribute to enhanced susceptibility or resistance to viral diseases. herein we review multiple discoveries made with genome-wide or candidate gene approaches that have revealed significant insights into virus-host interactions. genetic factors that have been identified include genes encoding virus receptors, receptor-modifying enzymes, and a wide variety of innate and adaptive immunity-related proteins. we d ... | 2017 | 28853921 |
| cytolytic virus activation therapy and treatment monitoring for epstein-barr virus associated nasopharyngeal carcinoma in a mouse tumor model. | undifferentiated nasopharyngeal carcinoma (npc) is 100% associated with epstein-barr virus (ebv). expression of viral proteins in the tumor cells is highly restricted. ebv reactivation by cytolytic virus activation (clva) therapy triggers de novo expression of early viral kinases (pk and tk) and uses antiviral treatment to kill activated cells. the mechanism of tumor elimination by clva was analyzed in npc mouse model using c666.1 cells. valproic acid (vpa) was combined with gemcitabine (gcb) to ... | 2017 | 28853217 |
| genomic analysis of nasopharyngeal carcinoma reveals tme-based subtypes. | nasopharyngeal carcinoma (npc) is an epstein-barr virus (ebv) associated cancer characterized by a poor prognosis and a high level of lymphocyte infiltrate. genetic hallmarks of npc are not completely known but include deletion of the p16 (cdkn2a) locus and mutations in nf-kb pathway components, with a relatively low total mutational load. to better understand the genetic landscape, an integrated genomic analysis was performed using a large clinical cohort of treatment-naïve npc tumor specimens. ... | 2017 | 28851814 |
| the impact of targeting repetitive bamhi-w sequences on the sensitivity and precision of ebv dna quantification. | viral load monitoring and early epstein-barr virus (ebv) dna detection are essential in routine laboratory testing, especially in preemptive management of post-transplant lymphoproliferative disorder. targeting the repetitive bamhi-w sequence was shown to increase the sensitivity of ebv dna quantification, but the variability of bamhi-w reiterations was suggested to be a source of quantification bias. we aimed to assess the extent of variability associated with bamhi-w pcr and its impact on the ... | 2017 | 28850597 |
| improved outcomes of kidney transplantation in infants (age < 2 years): a single center experience. | infants (age < 2 years) with end stage renal disease (esrd) have increased morbidity and mortality. we evaluated our long-term outcomes of kidney transplants (ktx) in infants. | 2017 | 28846558 |
| [high oncogenic risk human papillomavirus and urinary bladder cancer]. | to determine the role of human papillomavirus (hpv) of high oncogenic risk in the development of urinary bladder cancer. | 2017 | 28845940 |
| pediatric multiple sclerosis: genes, environment, and a comprehensive therapeutic approach. | pediatric multiple sclerosis is an increasingly recognized and studied disorder that affects 3%-10% of all patients. the risk for pediatric multiple sclerosis is thought to be secondary to a complex interplay between environmental and genetic risk factors. | 2017 | 28843454 |
| human immunodeficiency virus type 1 persistence following systemic chemotherapy for malignancy. | systemic chemotherapies for various malignancies have been shown to significantly, yet transiently, decrease numbers of cd4+ t lymphocytes, a major reservoir for human immunodeficiency virus type 1 (hiv-1) infection. however, little is known about the impact of cytoreductive chemotherapy on hiv-1 reservoir dynamics, persistence, and immune responses. | 2017 | 28838149 |
| valganciclovir for the suppression of epstein-barr virus replication. | epstein-barr virus (ebv) causes infectious mononucleosis and can lead to lymphoproliferative diseases. we evaluated the effects of valganciclovir on oral ebv shedding in a randomized, double-blind, placebo-controlled study. twenty-six men received oral valganciclovir or daily placebo for 8 weeks, followed by a 2-week "washout period" and then 8 weeks of the alternative treatment. valganciclovir reduced the proportion of days with ebv detected from 61.3% to 17.8% (relative risk, 0.28; 95% confide ... | 2017 | 28838145 |
| humanized mouse models for epstein barr virus infection. | it is essential for the human immune system to control epstein barr virus (ebv), because this common human γ-herpesvirus efficiently spreads through the human population with more than 90% being persistently infected after 20 years of age even in developed countries. moreover, it threatens each host with its potent growth transforming properties, readily immortalizing human b cells into persistently growing lymphoma cell lines. since this virus only infects humans, mice with reconstituted human ... | 2017 | 28837889 |
| concurrent chemoradiotherapy with or without cetuximab for stage ii to ivb nasopharyngeal carcinoma: a case-control study. | this study aimed to evaluate the long-term outcome and toxicities in patients with locoregionally advanced nasopharyngeal carcinoma (npc) treated by concurrent chemoradiotherapy (ccrt) with/without adding cetuximab. | 2017 | 28836950 |
| asymptomatic primary infection with epstein-barr virus: observations on young adult cases. | epstein-barr virus (ebv) is typically acquired asymptomatically in childhood. by contrast, infection later in life often leads to infectious mononucleosis (im), a febrile illness characterised by anti-ebv igm antibody-positivity, high loads of circulating latently-infected b cells, and a marked lymphocytosis caused by hyper-expansion of ebv-specific cd8(+) t cells plus milder expansion of cd56(dim) nkg2a(+) kir(-) nk cells. how the two situations compare is unclear due to the paucity of studies ... | 2017 | 28835490 |
| epstein-barr virus lmp1 mediated oncogenicity. | epstein-barr virus latent membrane protein 1 (lmp1) is expressed in multiple human malignancies, including nasopharynegeal carcinoma, hodgkin and immunosuppression-associated lymphomas. lmp1 mimics cd40 signaling to activate multiple growth and survival pathways, in particular nf-κb. lmp1 has critical roles in ebv-driven b-cell transformation, and its expression causes fatal lymphoproliferative disease in immunosuppressed mice. here, we review recent developments in studies of lmp1 signaling, lm ... | 2017 | 28835489 |
| ruxolitinib for secondary hemophagocytic lymphohistiocytosis: first case report. | hemophagocytic lymphohistiocytosis (hlh) is an immune-mediated disorder resulting in hyper-activation of inflammatory cytokines. if left untreated, the uncontrolled inflammatory response can lead to significant tissue injury and potentially life-threatening multi-organ dysfunction. conventional immunosuppressive agents are available for the management of hlh, including dexamethasone, cyclosporine, and etoposide; however, patients may not respond to these therapies. clinicians may turn toward alt ... | 2017 | 28834694 |
| evaluation of nasal and nasopharyngeal swab collection for the detection of epstein-barr virus in nasopharyngeal carcinoma. | ebv detection using nasopharyngeal swabs has been suggested as a potential screening test that could improve the specificity of current ebv-based serological assays. however, application requires insertion of the swab deep into the nasopharynx, a procedure not amenable to non-clinic screening. we reasoned that swabbing the more easily accessible nasal cavity might provide an appealing alternative for npc detection. | 2017 | 28833336 |
| long-term outcome of epstein-barr virus dnaemia and ptld with the use of preemptive rituximab following allogeneic hsct. | we studied retrospectively the outcome of epstein-barr virus (ebv)-related disease with ebv monitoring and preemptive rituximab to prevent post-transplant lymphoproliferative disorder (ptld) in 319 consecutive allogeneic stem cell transplantations 2004-2012. patients who received anti-thymocyte globulin (atg) or alemtuzumab were regarded as high-risk for ptld (n = 214). ebv dnaemia ≥1000 copies/ml plasma was observed in 50 (23%) of the high-risk patients. thirty-three of the high-risk (15%) and ... | 2017 | 28831836 |
| encephalitis with reversible splenial and deep cerebral white matter lesions associated with epstein-barr virus infection in adults. | approximately 200 cases of mild encephalitis with reversible splenial (mers) and deep cerebral white matter lesions have been reported since mers was first defined in 2004. mers occurs more frequently in children; in adults, only ~60 cases have been reported. until now, only four cases of mers in adults have been associated with epstein-barr virus (ebv). | 2017 | 28831257 |
| nasopharyngeal carcinoma super-enhancer-driven etv6 correlates with prognosis. | nasopharyngeal carcinoma (npc) most frequently occurs in southern china and southeast asia. epidemiology studies link npc to genetic predisposition, epstein-barr virus (ebv) infection, and environmental factors. genetic studies indicate that mutations in chromatin-modifying enzymes are the most frequent genetic alterations in npc. here, we used h3k27ac chromatin immune precipitation followed by deep sequencing (chip-seq) to define the npc epigenome in primary npc biopsies, npc xenografts, and an ... | 2017 | 28831010 |
| degradation of cofilin is regulated by cbl, aip4 and syk resulting in increased migration of lmp2a positive nasopharyngeal carcinoma cells. | expression of cofilin is directly associated with metastatic activity in many tumors. here, we studied the role of latent membrane protein 2 a (lmp2a) of epstein-barr virus (ebv) in the accumulation of cofilin observed in nasopharyngeal cancer (npc) tumor cells. we used lmp2a transformed npc cell lines to analyze cofilin expression. we used mutation analysis, ectopic expression and down-regulation of cbl, aip4 and syk in these cell lines to determine the effect of the lmp2a viral protein on cofi ... | 2017 | 28827787 |
| enhanced aerobic glycolysis of nasopharyngeal carcinoma cells by epstein-barr virus latent membrane protein 1. | latent membrane protein 1 (lmp1) is a principal viral oncoprotein in epstein-barr virus (ebv)-associated malignancies, including nasopharyngeal carcinoma (npc), which acts through regulating tumorigenesis and metabolic reprogramming of cancers. in the presence of oxygen, we demonstrated that glucose consumption, lactate production and lactate dehydrogenase (ldh) activity were significantly increased upon lmp1 expression in npc cells and in a lmp1 variant derived from npc patients-transformed bal ... | 2017 | 28827059 |
| spontaneous resolution of epstein-barr virus-induced haemophagocytic lymphohistiocytosis in still's disease treated by tocilizumab: a case report. | 2017 | 28826312 | |
| the complete remission of acquired immunodeficiency syndrome-associated isolated central nervous system lymphomatoid granulomatosis: a case report and review of the literature. | a 49-year-old man presented with gradually progressive aphasia one month after being diagnosed with acquired immunodeficiency syndrome (aids). brain magnetic resonance imaging showed multiple brain lesions with punctate and linear enhancement. a polymerase chain reaction detected epstein-barr virus (ebv) in the patient's cerebrospinal fluid. a diagnosis of isolated central nervous system lymphomatoid granulomatosis (cns-lyg) was made based on the brain biopsy findings. the complete remission of ... | 2017 | 28824078 |
| risk factors of lung cancer in nonsmoker. | generally, the cause of lung cancer is attributed to tobacco smoking. but many of the new lung cancer cases have been reported in nonsmokers. apart from smoking; air pollution, environmental exposure, mutations, and single-nucleotide polymorphisms are known to be associated with lung cancer. improper diet, alcohol consumption, marijuana smoking, estrogen, infections with human papillomavirus (hpv), hiv, and epstein-barr virus are suggested to be linked with lung cancer but clear evidences to asc ... | 2017 | 28823540 |
| [effect of complicated hemophagocytic syndrome on clinical prognosis of patients with non-hodgkin's lymphoma and analysis of its affecting factors]. | to investigate the effect of complicatal hemophagocytic syndrome on clinical prognosis of patients with non-hodgkin's lymphoma (nhl) and analyze its factors affecting prognosis. | 2017 | 28823265 |
| combined immunodeficiency with ebv positive b cell lymphoma and epidermodysplasia verruciformis due to a novel homozygous mutation in rasgrp1. | rasgrp1 is a guanine-nucleotide-exchange factor essential for map-kinase mediated signaling in lymphocytes. we report the second case of rasgrp1 deficiency in a patient with a homozygous nonsense mutation in the catalytic domain of the protein. the patient had epidermodysplasia verruciformis, suggesting a clinically important intrinsic t cell function defect. like the previously described patient, our proband also presented with cd4(+) t cell lymphopenia, impaired t cell proliferation to mitogen ... | 2017 | 28822832 |
| progression of understanding for the role of epstein-barr virus and management of nasopharyngeal carcinoma. | nasopharyngeal carcinoma (npc) is very common in southern china and southeast asia. in regions where npc is endemic, undifferentiated subtypes constitute most cases and are invariably associated with epstein-barr virus (ebv) infection, whereas the differentiated subtype is more common in other parts of the world. undifferentiated npc is a unique malignancy with regard to its epidemiology, etiology, and clinical presentation. clinically, npc is highly invasive and metastatic, but sensitive to bot ... | 2017 | 28819752 |
| corrigendum to "epstein-barr virus-induced gene 3 (ebi3) blocking leads to induce antitumor cytotoxic t lymphocyte response and suppress tumor growth in colorectal cancer by bidirectional reciprocal-regulation stat3 signaling pathway". | [this corrects the article doi: 10.1155/2016/3214105.]. | 2017 | 28819340 |
| how herpesviruses pass on their genomes. | herpesvirus genomes exist and replicate as episomes inside the host cell nucleus during latent infection. chiu et al. (2017. j. cell biol. https://doi.org/10.1083/jcb.201702013) find that unlike epstein-barr virus, which partitions viral genomes faithfully during cell division, kaposi's sarcoma-associated herpesvirus clusters viral genomes into loci that are distributed unequally to daughter cells. | 2017 | 28819012 |
| a french cohort study of kidney retransplantation after post-transplant lymphoproliferative disorders. | post-transplant lymphoproliferative disorders arising after kidney transplantation portend an increased risk of morbidity and mortality. retransplantation of patients who had developed post-transplant lymphoproliferative disorder remains questionable owing to the potential risks of recurrence when immunosuppression is reintroduced. here, we investigated the feasibility of kidney retransplantation after the development of post-transplant lymphoproliferative disorder. | 2017 | 28818847 |
| electromagnetic fields may act via calcineurin inhibition to suppress immunity, thereby increasing risk for opportunistic infection: conceivable mechanisms of action. | while a good number of studies have demonstrated that modern, man-made ambient electromagnetic fields can have both stimulatory and inhibitory effect on immune system function, the precise mechanisms have yet to be completely elucidated. it is hypothesized here that, depending on the parameters, one of the means by which long-term electromagnetic field exposure has the potential to eventually lead to immunosuppression is via downstream inhibition of the enzyme calcineurin - a protein phosphatase ... | 2017 | 28818275 |
| x-linked lymphoproliferative disease type 1 in a patient with the p.gly93asp sh2d1a gene mutation and hemophagocytic lymphohistiocytosis. | hemophagocytic lymphohistiocytosis is characterized by uncontrolled activation of the immune system that leads to systemic hyperinflammation. lymphoproliferative syndrome linked to the x chromosome is a hereditary immunodeficiency characterized by an inability to mount an adequate immune response to an epstein-barr virus infection. hemophagocytic lymphohistiocytosis is one of the main clinical features of x-linked lymphoproliferative syndrome. we report the case of a patient who presented with p ... | 2017 | 28816794 |