Rectal plasmablastic lymphoma in Ebstein Barr virus positive and human immunodeficiency virus negative subject after external radiation therapy for prostatic cancer

Plasmablastic lymphoma (PBL) represents a rare and aggressive subtype of diffuse large B cells lymphoma (DLBCL) most associated with the human immunodeficiency virus (HIV). Prognosis remains poor despite various treatment approaches. We describe an evolution at six months of HIV negative PBL and Ebstein Barr virus (EBV) positive PBL with chemotherapy. Role of radiotherapy is still unclear.

SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A CHILD WITH CONCOMITANT EBSTEIN BARR VIRUS AND SCRUB TYPHUS INFECTION – A RARE ENTITY

Scrub typhus is a life threatening zoonosis caused by Orientia tsutsugamushi organisms that are transmitted by the larvae of trombiculid mites. It is endemic to a geographically distinct region, the so called Tsutsugamushi triangle, which includes Japan, China and South Korea. The disease is more prevalent in southern and northern India.It is characterised by focal or disseminated vasculitis and perivasculitis involving the lungs, liver, spleen and central nervous system. We report our experience with pediatric scrub typhus at a hospital in eastern India with EBV positive and secondary HLH. An 8 year old boy with fever, maculopapular rash, hepatosplenomegaly and lymphadenopathy was admitted in our institution and diagnosed with Scrub typhus. Physical and laboratory data showed hepatosplenomegaly, bicytopenia, hyperferritinemia, and hypobrinogenemia. Secondary HLH was diagnosed and the child was managed with IVIG and steroids. In view of rash followed by lymphadenopathy and hepatosplenomegaly EBV serology was also sent as a cause of HLH. Surprisingly, EBV panel was also positive. We therefore concluded that the most probable explanation was EBV triggered HPS following scrub typhus infection. Another possible explanation is EBV can be reactivated in critically ill patients. To our knowledge this is the rst such case in the pediatric population reported till date.

Continuous Renal Replacement Therapy with oXiris® Membrane in Severe Ebstein-Barr Virus-Mediated Hemophagocytic Lymphohistiocytosis: A Case Report

Hemophagocytic lymphohistiocytosis (HLH), a life-threatening disease with uncontrolled immune activation and inflammatory reaction, often leads to a deadly cytokine storm. In severe Ebstein-Barr virus-triggered HLH receiving standard immunosuppression, continuous renal replacement therapy (CRRT) with oXiris^® blood purification membrane resulted in a timely reduction of inflammatory markers and discontinuation of vasopressors. To our knowledge, this is the first report of successful use of the oXiris^® membrane in HLH.

Viruses and esophageal cancer

Summary Esophageal adenocarcinoma (EAC) has had the fastest increasing incidence of any solid tumor in the United States in the last 30 years. Long standing gastroesophageal reflux disease is a well-established risk factor with strong associations with obesity, alcohol and tobacco. However, there are likely additional contributing factors. Viruses such as human papillomavirus, ebstein-barr virus and herpes simplex virus have been implicated in the pathogenesis of esophageal cancer. This review will discuss the known literature linking viruses to esophageal adenocarcinoma and consider future relationships such as identifying prognostic and predictive molecular biomarkers to guide therapies.

P5560Assessing etiology in a cohort of patients with myocarditis presenting with complex ventricular arrhythmias: can the percutaneous endomyocardial biopsy help?

Background Myocarditis represents a common but often under-diagnosed disease, with a wide range of clinical presentations; diagnosis is often presumptive and a clear etiology leading to a specific therapeutic approach is usually not identified. Purpose To describe and assess disease etiology in a cohort of myocarditis patients (pts) with arrhythmic presentation undergoing an invasive diagnostic work-up. Methods All pts with myocarditis presenting with ventricular arrhythmias undergoing an electro-anatomical mapping (EAM) guided endo-myocardial biopsy (EMB) at our institution were enrolled. All enrolled pts also underwent cardiac magnetic resonance imaging (MRI) and an electrophysiological study (EPS). Demographics, arrhythmic presentation, MRI data, arrhythmic inducibility at EPS, EAM and EMB biopsy data were retrieved and analyzed. Molecular biology testing for cardio-tropic virus genome as well as leukocyte immunohistochemical typization were routinely performed on all EMB samples. Results Twenty-six pts were enrolled (85% male, 39±6 y.o.). Clinical presentation was an organized ventricular arrhythmia in 16 (62%) pts (n=3 non-sustained ventricular arrhythmia; n=9 sustained ventricular arrhythmia; n=4 ventricular fibrillation) while frequent (>10.000) premature ventricular complexes (PVCs) in the remaining 10 (38%) pts. MRI showed a late gadolinium enhancement (LGE) pattern consistent with myocarditis in all pts (35% left LGE; 65% right LGE). At the EPS, 10 (38%) pts showed inducibility for SVTs and underwent an intra-cardiac defibrillator (ICD) implant, while 4 (16%) more were implanted for secondary arrhythmic prevention. EAM was performed in 18 (70%), 6 (22%) and 2 (8%) pts in the right, left and in both ventricle respectively; in all cases, abnormal myocardial voltages were retrieved in the area showing LGE at MRI. Extensive myocardial scarring was detected in 7 (27%) pts. All EMB were performed without peri-procedural complications; inflammatory infiltrate and substrate alteration consistent with myocarditis were retrieved in 100% of the bioptic samples. Viral genome was identified in 13 (50%) samples (n=5 Human Herpes Virus 6; n=2 Parvovirus B 19; n=3 Adenovirus; n=1 Ebstein Barr Virus; n=1 Cytomegalovirus; n=1 Rhinovirus) and specific human immunoglobulin treatment was undergone by a single pt; eosinophilic infiltration was found in 2 (8%) patients; lymphocite invasion and auto-antibodies consistent with auto-immune myocarditis were detected in 2 (8%) patients and appropriate immunosuppressive therapy was started, while a myocardial band contraction pattern typical of toxic myocarditis was found in a single (4%) patient [Figure 1]. Different Myocarditis Etiology Rates Conclusion In our myocarditis cohort, EMB confirmed viruses to represented the first myocarditis etiological agent. Despite an invasive work-out, 31% of the cohort etiology still remains unclear.

Nasopharyngeal Carcınoma in Chıldren and Adolescents; Experıence of EGE Unıversıty from Turkey

Nasopharynx cancer is a rare (3%) epithelial cancer which is seen in childhood and adolescence. It accounts for 30- 50% of the nasopharynx malignities at this age group. Incidence is high in China, South East Asia and Mediterranean countries. Genetic and geographic components play a major role in this high incidence. In these endemic regions, non-keratinized and undifferentiated carcinoma (type 2 and type 3) are histopathologically frequent and it is found that Ebstein – Barr virus (EBV) genes are related to the tumor. In our country, especially undifferentiated nasopharynx cancer is closely related to EBV infection. In childhood and adolescence, it is seen in localized advanced disease, also systemic disease characteristics are high. In recent years, primarily with cisplatine based chemotherapy protocol and appropriate doses of radiotherapy (RT), survival rates have reached 80-90%. In this retrospective study, our aim was to evaluate the clinical features, histophathology, treatment results and survival rates along with late toxicities of patients with NPC.