Presence of herpesviruses, parvoviruses, and polyomaviruses in sinonasal lymphoma.

PURPOSE: Sinonasal lymphoma (SL) is a rare lymphatic neoplasm of the nasal cavities, paranasal sinuses and nasopharynx. Whereas some risk factors for SL subtypes have been identified, their aetiology is unknown. Along with other predisposing factors, the viral association of lymphomas, such as Epstein-Barr virus (EBV) and Burkitt and Hodgkin lymphomas, is well-established. Modern molecular biology techniques have enabled the discovery of novel human viruses, exemplified by the protoparvovirus cutavirus (CuV), associated with cutaneous T-cell lymphoma. These findings, and the anatomical location of the sinonasal tract with its rich microbiome and infectious agents, justify in-depth studies among SL. METHODS: We analysed the presence of 20 viruses of Orthoherpesviridae, Parvoviridae, and Polyomaviridae by qPCR in 24 SL tumours. We performed RNAscope in situ hybridisation (RISH) to localize the viruses. Parvovirus-specific IgG was analysed by enzyme immunoassay and targeted next-generation sequencing (NGS) was applied to detect CuV in plasma. RESULTS: We detected viral DNA in 15/24 (63%) tumours; nine of EBV, six of human herpesvirus (HHV) -7, four each of HHV-6B and parvovirus B19, two of cytomegalovirus, and one each of CuV and Merkel-cell polyomavirus. We found tumours with up to four viruses per tumour, and localized CuV and EBV DNAs by RISH. Two of the ten plasma samples exhibited CuV IgG, and one plasma sample demonstrated CuV viremia by NGS. CONCLUSION: Viruses were frequent findings in SL. The EBV detection rate was high in diffuse large B-cell lymphoma, and co-detections with other viruses were prevalent.

[Clinicopathological features of primary EB virus positive nodal T/NK cell lymphoma].

Objective: To investigate the clinicopathological features of primary Epstein-Barrvirus (EBV) positive nodal T/NK-cell lymphomas (EBV+nodal TNKL). Methods: The clinicopathological features of 7 cases of EBV+nodal TNKL diagnosed between November 2015 and May 2019 at the First Affiliated Hospital of Zhengzhou University were analyzed using immunohistochemistry, PCR gene rearrangement and in situ hybridization.Follow-up data were also collected. Results: There were 5 males and 2 females with a median age of 54 years (ranged from 41 to 75 years). All patients presented with multiple lymphadenopathies and common B symptoms (5/7) and at an advanced Ann Arbor stage Ⅲ/Ⅳ(6/7). Bone marrow involvementwas detected in 1 patient.Six cases of T-cell origin had monomorphic patterns, and the tumor cells showed CD56 negativity and TCRαß+/TCRγδ－ with T-cell clonality. One case of NK-cell origin had polymorphic pattern, and the tumor cells showed CD56 positivity and TCRαß-/TCRγδ-without T-cell clonality. All cases were positive for the cytotoxic markers, but showed various CD4/CD8 expression. All 7 cases were diffusely positive for EBV (>100 cell/high power field). Six of the patients received chemotherapy, and 1 patient declined the treatments. During the follow-up period ranging from 3 to 48 months, 5 of the 7 patients died of the disease. Conclusions: EBV+nodal TNKL is a rare entity and is characterized by cytotoxic molecule expression, T/NK-cell derivation, and a predominance of nodal involvement at an advanced stage. It should be differentiated from other EBV+T/NK cell lymphoproliferative disorders, especially extranodal NK/T cell lymphoma.

[Clinicopathological features of fibrin-associated diffuse large B cell lymphoma arising within concurrent atrial myxoma: report of six cases].

Objective: To investigate the clinicopathological features, treatment and prognosis of fibrin-associated diffuse large B cell lymphoma (DLBCL) arising within concurrent atrial myxoma. Methods: Six cases of fibrin-associated DLBCL arising within concurrent atrial myxoma diagnosed at the Department of Pathology, Guangdong General Hospital, from 2006 to 2019 were included. The histology, immunophenotype, treatment and prognoses were analyzed. Results: The patients' age ranged from 46 to 78 years (mean 59 years). There were 3 males and 3 females. The tumors were all discovered incidentally on histological examination of surgical pathology specimens excised for atrial myxoma. All patients appeared to have morphological features of DLBCL, B lineage immunophenotype, high proliferative index and latency type III of Epstein-Barr viral infection. They had complete tumor resections without adjuvant chemotherapy and were healthy at 5- to 120-month follow-ups. Conclusions: Fibrin-associated DLBCL arising within concurrent atrial myxoma is an unusual form of DLBCL associated with chronic inflammation, and its clinical outcome is indolent. The findings suggest that this type of lymphoma does not warrant excessive or unnecessary treatments after complete resection.

[Relationship between morphological characteristics and prognosis of non-nasopharyneal EBV-associated carcinoma].

Objective: To analyze the pathological features and their influence on the clinical outcome of non-nasopharyngeal EBV-associated carcinomas. Methods: One hundred and twenty cases of non-nasopharyngeal EBV-associated carcinoma confirmed by in situ hybridization were identified at Zhejiang Cancer Hospital from January 1, 2006 to May 1, 2018, and the clinicopathological data were collected and analyzed using Kaplan-Meier survival analysis, Cox univariate and multivariate analysis. Results: One hundred and twenty cases were involved in the study; the male to female ratio was 1∶1; patients' age range was 24 to 89 years (median 50 years). The primary sites were large parotid glands (62 cases), lung(26 cases), stomach(15 cases), and others (oral, oropharynx, larynx, cervix, liver; totally 17cases). Non-nasopharyngeal EBV-associated cancer could be divided into two histological types according to the amount of interstitial lymphocytes: type Ⅰ was "lymphoepithelial-like carcinoma" and rich in stromal lymphocytes; type Ⅱ lacked lymphocytic infiltration. Ninety-eight primary tumor samples could be classified morphologically: 43 cases were as type Ⅰ and 55 cases as typeⅡ; the distribution of type Ⅰ was 57.4% (27/47) in large parotid glands, 20.8% (5/24) in lung, 4/13 in stomach, and 7/14 in other sites. Complete treatment and survival data were obtained for 114 patients. According to the TNM staging criteria of WHO, 52 patients were at early stages (Ⅰ-Ⅱ) and 62 were at advanced stages (Ⅲ-Ⅳ); 102 patients underwent surgery. Seventy-four patients received adjuvant chemotherapy before or after surgery, and 52 patients received local radiotherapy. Kaplan-Meier survival analysis showed that patients with type Ⅱ EBV-associated carcinoma had a worse prognosis than patients with type Ⅰtumors (P=0.010 2). In addition, vascular invasion(P=0.021 8),neural recidivism(P=0.000 1),advanced stage(P=0.017 1),lymph node metastasis (P=0.005 0) and chemotherapy (P=0.013 2) were poor prognostic factors; female patients had better survival than male (P=0.028 4). Cox multivariate regression analysis found that lymph node metastasis (95%CI: 1.489-13.830, P=0.007 6) and neural recidivism (95%CI: 1.228-6.544, P=0.014 7) were independent adverse prognostic factors. Cox multivariate regression analysis after stratification by site revealed that radiotherapy was a preferable prognostic factor for EBV-associated carcinoma of the large salivary glands (95%CI: 0.003-0.569, P=0.016 8). Conclusion: EBV associated carcinoma can be divided into two types, for which type Ⅰ was with abundant interstitial lymphocytes and type Ⅱ was lack of interstitial lymphocytes. TypeⅡ EBV-associated carcinoma has a worse prognosis than type Ⅰ. Radiation therapy can prolong the survival time of patients with primary EBV-associated carcinoma of large salivary glands.

[EB virus-positive T/NK lymphoproliferative diseases: an analysis of 156 patients].

Objective: To investigate the clinicopathological features of EBV-positive T/NK cell lymphoproliferative diseases (EBV(+) T/NK-LPD). Methods: The clinical characteristics of 156 cases of EBV(+) T/NK-LPD were collected from August 2002 to March 2015 at Beijing Friendship Hospital, Capital Medical University. Immunohistochemical staining, EBER in situ hybridization and clonal analysis of TCR gene were performed. All patients were followed up. Results: There were 106 male and 50 female patients; patients' age ranged from 1 to 75 years (median 20 years). The course of the diseases before diagnosis ranged from 2 to 540 months (median 20 months). Fever was noted in 122 patients (78.2%), 108 patients had lymphadenopathy (69.2%), and 75 patients had hepatosplenomegaly (48.1%). Thirty-three cases were grade 1, 68 cases were grade 2, and 55 cases were grade 3. TCR gene arrangement analysis was performed in 45 cases, and 33 cases (73.3%) showed clonal rearrangement. The follow-up period ranged from 1-134 months, and 44 patients (28.2%) died. There was a trend of increased death rate associated with increasing grade (P>0.05). Conclusions: There are many types of EBV(+) T/NK-LPD, and they can be classified as systemic, relatively localized and localized. The prognosis should be based on a comprehensive analysis of pathology and clinical data. There is no significant correlation between morphological grade and mortality. An important goal of therapy is to prevent serious complications.

[Clinicopathologic features of primary mucosal CD30-positive T-cell lymphoproliferative disorders in head and neck region].

Objective: To study clinicopathologic features, prognosis and differential diagnoses of primary mucosal CD30-positive T-cell lymphoproliferative disorders of the head and neck(mCD30(+) TLPD-head and neck). Methods: Three cases of mCD30(+) TLPD-head and neck were collected from January 2014 to April 2017 at Sun Yat-Sen University Foshan Hospital. A literature review of mCD30(+) TLPD of head and neck was provided. Results: All three cases presented with either bulging/exophytic nodule or mucosal ulcer/erosion. Morphologically, the tumor consisted of diffuse proliferation of uniform, large atypical mononuclear lymphoid cells that showed irregular or polymorphic nuclei with small nucleoli, and abundant pale or amphophilic cytoplasm. Hallmark cells with eccentric, horseshoe, kidney-like, or doughnut-shaped nuclei were present. While mitotic figures were present, no tumor necrosis was found. Eosinophilc infiltration was obvious in the background. The atypical large lymphoid cells had a immunophenotype of CD30(+) /CD3(+) /CD4(+) /CD56(-) along with positive cytotoxic molecule. While being negative for EBER/ALK/CD20/CD8, TCR rearrangement was found in 2 out of 3 cases. Three patients were cured after excision without relapse and metastasis.The two patients with TCR rearrangement didn't show aggressive clinical course. Conclusions: mCD30(+) TLPD-head and neck is a rare benign lymphoproliferative disorder with spontaneous regression. It should be differentiated from cutaneous CD30(+) anaplstic large cell lymphoma, lymphomatoid papulosis, and EBV-related mucocutaneous ulcer. Correct recognition of mCD30(+) TLPD of head and neck is important to avoid overtreatment.

[Expression of long non-coding RNA SNHG8 in Epstein-Barr virus-related gastric cancer and clinical outcome].

Objective: To investigate the expression of long non-coding RNA (lncRNA) SNHG8 in EB virus related gastric cancer and their correlation prognosis. Methods: The expression of SNHG8 in 93 gastric cancers and 93 cancer-free controls, matched by age and sex, were determined by real-time PCR. EB virus expression was detected by EBER in situ hybridization. Results: Forty-one gastric cancers were EB virus associated. For all gastric cancers, SNHG8 expression was 14 times higher (P=0.001) than that in non-cancer controls; in the EB virus related gastric cancers, SNHG8 expression was increased 25 times (P<0.05) over EB virus negative gastric cancers. SNHG8 expression level was also significantly associated with TNM staging (P<0.05). Conclusions: SNHG8 may act as a proto-oncogene, participating in gastric carcinogenesis.EB virus infection of gastric mucosa may promote SNHG8 expression.

Epstein - Barr Virus Salivary Shedding in Patients with Acute Infectious Diseases: A Pilot Study.

Background: Epstein-Barr virus (EBV) is a widely disseminated herpesvirus for which antibodies have been demonstrated in over 90% of adults worldwide. After subclinical primary EBV infections, as well as after infectious mononucleosis, the virus can be shed in saliva for a prolonged period of time. Aim: Diseases and disorders that can induce EBV salivary shedding include mental disorders and sex, connective tissue disease, multiple sclerosis, systemic lupus erythematosus, malaria and HIV infection. Since the occurrence of EBV in saliva during acute infectious diseases has not yet been systematically researched, we aimed to investigate the possible relationship between acute infectious diseases and salivary shedding of EBV. Material and methods: This pilot cross-sectional study included consenting adults hospitalized for acute infectious conditions and their peers free of acute infectious diseases. A total of 40 patients with acute infectious diseases were enrolled, along with 41 adults free of acute infections. Peripheral venous blood samples for serodiagnosis and saliva samples for EBV PCR testing were collected from both groups. We fitted logit and general linear models to proportions and to ln (viral copy counts) to generate adjusted proportions and geometric mean values in the two groups of subjects. We used SAS for Windows 9.4. Results: The most common acute infectious disease was COVID-19 pneumonia, followed by hemorrhagic fever with renal syndrome. Crude proportions of people with positive serological test results and those with saliva viral shedding were similar in the two groups. Conclusions: The presented preliminary data do not indicate acute infectious conditions as a marked "contributor" in increasing salivary EBV shedding.

Extranodal NK/T cell lymphoma, nasal type: An updated overview.

Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is an aggressive malignancy associated with Epstein-Barr virus infection, with a geographic and racial predilection for some Asian and Latin American countries. ENKTCL-NT manifests as a necrotic process affecting nasal or upper aerodigestive structures and, rarely, extranasal sites such as skin, and the gastrointestinal tract. ENKTCL-NT was characterized by its poor prognosis irrespective of clinical stage and therapy. However, during the last two decades, advances in its clinicopathologic, genetic and molecular characterization have been achieved, as have changes in the chemotherapy regimens that, in combination with radiotherapy, are significantly improving the survival of these patients, especially in initial stages. For these reasons, we present an overview of the historical background of ENKTCL-NT along with an updated review of its potential etiological factors, clinicopathologic and molecular features, as well as its prognostic models, current treatment protocols, and future directions on potential promising therapeutic approaches.

[Clinical and pathological features of 13 children with Epstein-Barr virus-positive lymphoproliferative disease].

Objective: To summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease (EBV(+)LPD) in children. Methods: The clinical and follow-up data of 13 children histopathologically diagnosed as EBV(+)LPD in the Department of Infectious Disease of Beijing Children's Hospital between January 2011 and December 2016 were summarized. Results: Of the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years (range 1.3 to 15.0 years). The median duration between disease onset and diagnosis was 3 months (range 1 to 24 months). All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×10(8)/L to 1×10(11)/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease(EBV(+) T-LPD), 2 cases with hydroa vacciniforme (HV) and 2 cases with EBV-positive diffuse large B-cell lymphoma (EBV(+) DLBCL) . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV(+)T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis(HLH) 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still have symptoms. Among 2 cases of EBV(+) DLBCL, 1 case died shortly after discharge and the other was still stable after chemotherapy. Conclusions: Chronic recurrent fever, lymphadenopathy and hepatosplenomegaly are the most common clinical manifestations in children with EBV(+)LPD. Involvement of lung, central nervous system, intestinal tract, skin and other organs are also involved frequently. For children with chronic fever of unknown cause and accompanied by lymphadenopathy and (or) hepatosplenomegaly, EBV (+) LPD should be considered highly when the whole blood EBV-DNA load continues to increase significantly, early biopsy of the proliferative lesion should be performed to make a definite diagnosis. The prognosis of EBV (+) LPD is poor, and some evolve to lymphoma, hematopoietic stem cell transplantation is an effective way to treat this disease.

Epstein-Barr virus in gastric cancer and association with 30 bp del-latent membrane protein 1 polymorphism

SUMMARY OBJECTIVE: This study aimed to determine the frequencies of Epstein-Barr virus, types 1 and 2 infection, and 30 bp del-latent membrane protein 1 viral polymorphism in gastric adenocarcinomas, as well as to investigate the association between Epstein-Barr virus infection and tumor location, type, and the patient's sex. METHODS: Samples were collected from 38 patients treated at a university hospital in Rio de Janeiro, Brazil. Epstein-Barr virus detection and genotyping were performed by polymerase chain reaction, followed by polyacrylamide gel electrophoresis and staining by the silver nitrate method. RESULTS: Overall, 68.4% of patients had Epstein-Barr virus-positive tumors. Of these, 65.4% presented infection by Epstein-Barr virus type 1, 23.1% by Epstein-Barr virus type 2, and 11.5% had coinfection with types 1 and 2. The 30 bp del-latent membrane protein 1 polymorphism was found in 42.3% of Epstein-Barr virus-positive tumors, 23.1% had the wild-type virus, and 23.1% had the wild-type and the polymorphism concomitantly. In 11.5% of Epstein-Barr virus-positive tumors, it was impossible to determine whether there was polymorphism or not. Tumor location in the antrum (22 of 38) and diffuse type (27 of 38) were predominant. There was no significant difference in Epstein-Barr virus infection or the 30 bp del-latent membrane protein 1 polymorphism between men and women. CONCLUSION: Epstein-Barr virus infection was found in 68.4% of tumors investigated in this study. To the best of our knowledge, this is the first article showing the coinfection of Epstein-Barr virus types 1 and 2 in gastric carcinoma in Brazil.

[Causes and management of pediatric spontaneous tonsillar haemorrhage].

Objective: To analyze the causes and management plan of pediatric spontaneous tonsillar haemorrhage(STH). Methods: According to the criteria of STH difined by Griffies, patients with STH from December 2013 to January 2016 were included in this retrospective study. Results: A total of 11 patients were reviewed. The etiological diagnosis included 3 pediatric Epstein-Barr virus associated infectious mononucleosis(EBV-IM), 3 suspected pediatric EBV-IM to 3 acute suppurative tonsillitis, 1 acute viral tonsillitis and 1 hemophilia A. The management strategies included antiviral, antibacteria, transfusion, surgical examination followed with bipolar coagulation hemostasis under general anesthesia. No patient treated with tosillectomy. Conclusions: STH is now a rare condition, the causes of which in child are more or less different from adult.This emergency can be treated in time if a detailed management plan for pediatric STH is formulated.

Lymphoepithelioma-like carcinoma of the uterine cervix.

Lymphoepithelioma-like carcinoma (LELC) of the uterine cervix is exceedingly uncommon. We herein report a rare case of cervical LELC. A 45-year-old woman was admitted to gynecology department with vaginal bleeding for one month. Liquid-based cytology revealed atypical endometrial cells, not otherwise specified on her cervix. On a hysteroscopy, an endocervical mass was identified and the pathologic result was consistent with poorly differentiated squamous cell carcinoma. Magnetic resonance imaging and positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography revealed a 3.1-cm endocervical mass without distant metastasis or enlarged lymph nodes. The International Federation of Gynecology and Obstetrics stage was IB1. A radical hysterectomy and bilateral pelvic lymph node dissection were performed. The pathologic diagnosis was a poorly differentiated carcinoma, showing features of LELC. She has been followed for 8 months without adjuvant treatment since the surgery, during which time there has been no evidence of tumor recurrence or metastasis.

Herpes Group Viruses: a Seroprevalence Study in Hemodialysis Patients.

Herpes group viruses (herpes simplex virus, HSV; varicella-zoster virus, VZV; cytomegalovirus, CMV; and Epstein-Barr virus, EBV) remain an important cause of morbidity in immunocompromised persons. The aim of the study was to analyze the prevalence of HSV-1, HSV-2, VZV, CMV and EBV in patients undergoing hemodialysis. During a three-year period (2013-2015), 152 consecutive serum samples from hemodialysis patients and 150 healthy subjects (control group) were tested for the presence of IgM/IgG antibodies to herpes group viruses. Serologic tests were performed using a commercial enzyme-linked immunosorbent assay (ELISA) or enzyme-linked immunofluorescent assay (ELFA). Hemodialysis patients showed significantly higher CMV IgG seropositivity compared to controls (88.2% vs. 78.7%, p=0.011). In addition, seroprevalence rates of HSV-1 and VZV were higher in hemodialysis patients; however, these differences did not reach statistical significance (85.5% vs. 80.0%, p=0.054 and 99.3% vs. 96.0%, p=0.051, respectively). The prevalence of HSV-2 and EBV was similar in both groups (12.5% vs. 12.7%, p=0.137 and 98.0% vs. 95.3%, p=0.113, respectively). There was no difference in IgG seropositivity according to gender and place of residence. Logistic regression showed that older age was a significant predictor for CMV and EBV IgG seropositivity (increase in age by one year: CMV OR=1.055; 95%CI=1.030-1.080 and EBV OR=1.075, 95%CI=1.023-1.130).

Treatment-related fluctuations in Guillain-Barré syndrome​: clinical features and predictors of recurrence / Fluctuaciones relacionadas al tratamiento en el Síndrome de Guillain-Barré: características clínicas y predictores de recurrencia

ABSTRACT Background: A treatment-related fluctuation (TRF) in a patient with Guillain-Barré syndrome (GBS) is defined as clinical deterioration within two months of symptom onset following previous stabilization or improvements with treatment. Objective: To investigate the clinical characteristics and factors that could increase the risk of relapse of GBS in patients with and without TRFs. Methods: Retrospective review of medical records of patients (>18 years) with GBS evaluated between January/2006 and July/2019. Demographic and clinical characteristics, ancillary studies, treatment received, and the clinical course of patients with and without TRFs were analyzed. Results: Overall, 124 cases of GBS were included; seven (5.6%) presented TRFs. GBS-TRF cases were triggered more frequently by infectious mononucleosis (28.57 vs. 8.55%; p=0.01). GBS-TRF were initially treated with plasmapheresis more frequently than those without TRF (14.29 vs. 1.70%; p=0.0349). Combined treatment (71.43 vs. 4.27%; p<0.001) and corticosteroids (42.86 vs. 1.71%; p<0.001) were more commonly used in the GBS-TRF group. GBS-TRF patients presented a higher median initial disability score (4 vs. 2; p=0.01). Conclusions: Patients with GBS triggered by infectious mononucleosis and a high degree of initial disability have higher chances of developing TRFs. Although patients with TRF were treated with plasmapheresis more often, the total number was too low to suggest a link between plasma exchange and TRF.

RESUMEN Antecedentes: Una fluctuación relacionada al tratamiento (FRT) en un paciente con síndrome de Guillain-Barré (SGB) se define como un deterioro clínico dentro de los dos meses posteriores al inicio de los síntomas después de una estabilización previa o mejoría con el tratamiento. Objetivo: Investigar las características clínicas y los factores que podrían incrementar el riesgo de recaída, comparando pacientes con SGB, con y sin FRT. Métodos: Revisión retrospectiva de historias clínicas de pacientes (>18 años) con SGB evaluados entre enero/2006 y julio/2019. Se analizaron las características demográficas y clínicas, los estudios complementarios, el tratamiento recibido y la evolución clínica de los pacientes con y sin FRT. Resultados: Se incluyeron 124 casos de SGB en el total; 7 (5,6%) presentaron FRT. Los casos de SGB con FRT se desencadenaron con mayor frecuencia por mononucleosis infecciosa (28,57 vs. 8,55%; p=0,01). Los casos de SGB con FRT se trataron inicialmente con plasmaféresis con más frecuencia que aquellos sin FRT (14,29 vs. 1,70%; p=0,0349). El tratamiento combinado (71,43 vs. 4,27%; p<0,001) y los corticosteroides (42,86 vs. 1,71%; p<0,001) se utilizaron con mayor frecuencia en el grupo de SGB con FRT. Los pacientes con FRT presentaron una escala de discapacidad inicial mediana más alta (4 vs. 2; p=0,01). Conclusiones: Aquellos SGB desencadenados por mononucleosis infecciosa y un alto grado de discapacidad inicial tienen una mayor probabilidad de desarrollar FRT. Aunque los pacientes con FRT fueron tratados con plasmaféresis con mayor frecuencia, el número total fue demasiado bajo para sugerir un vínculo entre la plasmaféresis y FRT.

A French Cohort Study of Kidney Retransplantation after Post-Transplant Lymphoproliferative Disorders.

BACKGROUND AND OBJECTIVES: 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. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We reviewed the data from all patients who underwent kidney retransplantation after post-transplant lymphoproliferative disorder in all adult kidney transplantation centers in France between 1998 and 2015. RESULTS: We identified a total of 52 patients with kidney transplants who underwent 55 retransplantations after post-transplant lymphoproliferative disorder. The delay from post-transplant lymphoproliferative disorder to retransplantation was 100±44 months (28-224); 98% of patients were Epstein-Barr virus seropositive at the time of retransplantation. Induction therapy for retransplantation was used in 48 patients (i.e., 17 [31%] patients received thymoglobulin, and 31 [57%] patients received IL-2 receptor antagonists). Six patients were also treated with rituximab, and 53% of the patients received an antiviral drug. The association of calcineurin inhibitors, mycophenolate mofetil, and steroids was the most common maintenance immunosuppression regimen. Nine patients were switched from a calcineurin inhibitor to a mammalian target of rapamycin inhibitor. One patient developed post-transplant lymphoproliferative disorder recurrence at 24 months after retransplantation, whereas post-transplant lymphoproliferative disorder did not recur in 51 patients. CONCLUSIONS: The recurrence of post-transplant lymphoproliferative disorder among patients who underwent retransplantation in France is a rare event.

Extranodal nk/t-cell lymphoma, nasal type: case report

ABSTRACT Introduction: Extranodal NK/T-cell lymphoma, nasal type (ENKL), is a highly aggressive non-Hodgkin's lymphoma of unknown etiology. Clinical manifestations are usually nasal obstruction, epistaxis, and inflammatory signs. Diagnosis can be difficult to achieve and requires histo-pathology and immunohistochemistry studies. Radiotherapy and chemotherapy have been proposed as treatment, depending on the stage of the disease. Case presentation: A 44-year-old male patient, previously healthy, attended the outpatient service due to clinical nasal obstruction, secretion, edema, and facial erythema for 2 months. Facial cellulitis was initially diagnosed, but since there was no improvement with antibiotic treatment, the patient was referred to the emergency department of a higher complexity center, where complementary imaging showed a mass in the right nasal cavity. A histopathological analysis established that it was an ENKL, so radiotherapy was indicated; however, as a result of the advanced stage of this neoplasm, the patient died. Conclusion: Timely diagnosis of ENKL is crucial to improve life expectancy. Nevertheless, it may represent a clinical challenge due to its nonspecific presentation.

RESUMEN Introducción. El linfoma extranodal nasal de células T/natural killer (ENKL) es un linfoma no Hodgkin altamente agresivo y de etiología desconocida. Sus manifestaciones clínicas suelen ser obstrucción nasal, epistaxis y signos inflamatorios; sin embargo, el diagnóstico puede llegar a ser difícil, requiriendo histopatología e inmunohistoquímica para su detección. Como tratamiento se han propuesto radioterapia y quimioterapia, según el estadio de la enfermedad. Presentación del caso. Paciente masculino de 44 años de edad, previamente sano, quien asistió al servicio de consulta externa por cuadro clínico de 2 meses de evolución consistente en síntomas de obstrucción y secreción nasal, edema y eritema facial. Se realizó diagnóstico inicial de celulitis facial, pero dado que no hubo mejoría con el tratamiento antibiótico, el sujeto fue remitido al servicio de urgencias de una institución de mayor complejidad, donde se le practicaron imágenes complementarias que mostraron una masa en la cavidad nasal derecha; mediante análisis histopatológico se estableció que se trataba de un ENKL, por lo que se indicó radioterapia; sin embargo, a consecuencia del estadio avanzado de dicha patología, el paciente falleció. Conclusión. El diagnóstico oportuno de ENKL es crucial para mejorar la expectativa de vida de quienes lo padecen; sin embargo, puede representar un reto clínico debido a su presentación inespecífica.

Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr / Hemophagocytic syndrome, current diagnostic and therapeutic approach

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.

Síndrome Hemofagocítico en Niños Reporte de Caso / Hemophagocytic Syndromein children: a case report

Introducción:El síndrome hemofagocítico (SHF) es reconocido como un conjunto de signos clínicos y hallazgos laboratoriales que tienen un grave compromiso en la salud y vitalidad de los niños con una incidencia de 1.2 casos/millón/año. Puede pasar subdiagnosticado y confundido con sepsis de foco inespecífico Caso clínico:Niño de 4 años de edad, sin antecedentes de importancia. Ingresado desde el servicio de emergencia por presentar 20 días de fiebre y dolor abdominal. Requirió intubación por franca falla respiratoria y el ingreso a la Unidad de Cuidados Intensivos Pediátricos. Con hipotensión e insuficiencia hepática, pancitopeniay esplenomegalia. Evolución: Se descartaron infecciones bacterianas con policultivos, SARS-Cov 2negativo,se descartaron inmunodeficiencias congénitas y adquiridas.TORCHnegativo, VDRL no reactivo.La prueba de Epstein Barr fue positivo para IgM.Se determinó endocarditis con derrame pericárdico global. Estudio de biopsia medular normocromía, normocitosis, pancitopenia y blastos <5%, sin infiltración tumoral. Se estableció el Diagnóstico de SHFse inicióciclosporina y corticoterapia.Requirió ventilación mecánica por 20 días con período de pronación de 36 horas. Fue dado de alta a pediatríay posteriormente a domicilio, para control por consulta externa. Conclusión: El diagnóstico del SHF es inusual y subestimado al momento de la evaluación clínica. En el presente reporte se asocia a la presencia del Virus Epstein Barr

Introduction: Hemophagocytic syndrome (HPS) is recognized as a set of clinical signs and laboratory findings that have a serious compromise on the health and vitality of children with an incidence of 1.2 cases / million / year. It can be underdiagnosed and confused with sepsis with a non-specific focus. Clinical case: 4-year-old boy, with no significant history. Admitted from the emergency service due to 20 days of fever and abdominal pain. She required intubation due to frank respiratory failureand admission to the Pediatric Intensive Care Unit. With hypotension and liver failure, pancytopenia and splenomegaly. Evolution: Bacterial infections were ruled out with polycultures, SARS-Cov 2 negative, congenital and acquired immunodeficiencies were ruled out. Negative TORCH, non-reactive VDRL. The Epstein Barr test was positive for IgM. Endocarditis with global pericardial effusion was determined. Medullary biopsy study normochromia, normocytosis, pancytopenia, and blasts <5%, without tumor infiltration. The diagnosis of SHF was established, cyclosporine and corticosteroid therapy were started. He required mechanical ventilation for 20 days with a 36-hour pronation period. He was discharged to pediatrics and later at home, for outpatient control. Conclusion: The diagnosis of HHS is unusual and underestimated at the time of clinical evaluation. In this report it is associated with the presence of the Epstein Barr Virus