Chronic active EBV infection: the experience of the Samsung Medical Center in South Korea

AbstractBackgroundChronic active EBV infection (CAEBV) of T-cell or NK-cell type is an EBV+ polyclonal, oligoclonal or often monoclonal lymphoproliferative disorder (LPD) recognized as representing the spectrum of EBV-associated T-cell and NK-cell LPD with different clinical presentations; one systemic and two cutaneous disorders including hydroa vacciniforme-like T-cell LPD and mosquito bite hypersensitivity. The systemic form of the disease is characterized by fever, persistent hepatitis, hepatosplenomegaly and lymphadenopathy, which shows varying degrees of clinical severity depending on the immune response of the host and the EBV viral load.Case reportsWe described the clinicopathological findings of two children with CAEBV with a brief review of the literature.ConclusionsRecognition of the disease is important for adequate management of the patient. EBV analysis should be included in the principal diagnostic tests for febrile children

Epstein-Barr virus-positive T/NK-cell lymphoproliferative diseases in children and adolescents

In primary Epstein-Barr virus (EBV) infection, the virus may infect T-cells or natural killer (NK)-cells and can cause T- and NK-cell (T/NK-cell) lymphoproliferative disease, which encompasses several disease entities with a broad clinicopathological spectrum. T/NK-cell lymphoproliferative disease arising during primary EBV infection includes clonal EBV-infected T-cell proliferation in the setting of infectious mononucleosis, EBV-associated hemophagocytic lymphohistiocytosis, and systemic T-cell lymphoma of childhood. Chronic active EBV (CAEBV) infection of the T/NK-cell type is not a simple viral infection but instead is classified as a lymphoproliferative disease. The prototype of CAEBV is a systemic form that exhibits varying degrees of clinical severity depending on the host immunity and EBV factor. Hydroa vacciniforme-like lymphoproliferative disease and mosquito-bite allergy are peculiar cutaneous forms of CAEBV that involve T/NK-cells, respectively. Abnormal activation and replication of EBV together with the proliferation and clonal expansion of infected cells, depending on the patient’s immunological response, play a key role in the pathogenesis of CAEBV of the T/NK-cells type

Systemic EBV+ T-cell lymphoma in elderly patients: comparison with children and young adult patients

Fulminant Epstein–Barr virus (EBV+) T-cell lymphoma in immunocompetent elderly patients is rare and its character has not been well defined. This study analyzed the clinicopathological features of five elderly patients (group A: 50–84 years) and compared them with those of eight children and young adult patients with systemic T-cell lymphomas (group B: 10–34 years). Group A more commonly presented with generalized lymphadenopathy (n = 3) than did group B (n = 1). Chronic active EBV infection (n = 3) and hydroa vacciniforme-like eruptions (n = 1) were seen in group B, while group A showed no evidence of chronic EBV infection, but did show chronic hepatitis B or C virus infections (n = 3). The histological and immunophenotypical findings were similar. All patients died within 1 to 14 months of diagnosis. These findings suggest that EBV+ T-cell lymphoma in elderly patients is a unique disease with an underlying derangement of T-cell immunity and failure to eradicate infected virus. Additional factors related to senility may play a role in the disruption of homeostasis between the virus and the host’s immune system

The Clinical Usefulness of 18F-FDG PET/CT for the Evaluation of Lymph Node Metastasis in Periorbital Malignancies

PURPOSE: Surgical treatment of malignancies in the oral cavity and subsequent radiotherapy often result in an oral condition unfavorable for prosthodontic rehabilitation. This study assessed the quality of life related to oral function in edentulous head and neck cancer patients following oncology treatment of malignancies in the lower region of the oral cavity. MATERIALS AND METHODS: Patients treated between 1990 and 2000 with surgery and radiotherapy for a squamous cell carcinoma in the oral cavity who were edentulous in the mandible and had been treated with a conventional, non-implant-retained denture received an invitation for a clinical check-up (clinical assessment, questionnaires regarding oral function and quality of life). RESULTS: Sixty-seven of the 84 patients who fulfilled the inclusion criteria were willing to participate in the study. The mean irradiation dosage that these patients had received in the oral region was 61.8 +/- 5.4 Gy. Half of the patients (n=33) were not very satisfied with their prostheses; they wore their mandibular prostheses at most a few hours per day. It was concluded from the clinical assessment that two thirds of the patients (n 4) could benefit from an implant-retained mandibular denture. Analyses of the questionnaires revealed no significant associations between functional assessments, quality of life, and parameters such as size of the primary tumor, location of the primary tumor, and different treatment regimes. Despite cancer treatment, the patients reported a rather good general quality of life. CONCLUSIONS: Sequelae resulting from radiotherapy probably dominate oral function and quality of life after oncology treatment. In two thirds of the patients, improvement of oral function and related quality of life would be expected with the use of an implant-retained mandibular denture

Aggressive natural killer cell leukemia: Therapeutic potential of l-asparaginase and allogeneic hematopoietic stem cell transplantation

We conducted a retrospective JapanKorea multicenter study to better elucidate the clinicopathologic features and therapeutic modalities for aggressive natural killer cell leukemia (ANKL). A total of 34 patients were analyzed. The median age of the patients was 40 years. Among the patients in the study, four had a history of EpsteinBarr virus-related disorders. Three types of ANKL cells were categorized according to their morphological features. Leukemic cells were below 20% in both peripheral blood and bone marrow of 11 patients. The clinical characteristics and prognoses of these 11 patients did not differ significantly from those of the others. As an initial therapy, l-asparaginase chemotherapy resulted in a better response. A total of six patients received allogeneic hematopoietic stem cell transplantation (HSCT) and two received autologous HSCT, with all in non-complete remission (CR). After HSCT, four with allogeneic and one with autologous HSCT reached CR. Median survival of all patients was 51 days. Median survival for the patients with and without HSCT were 266 and 36 days, respectively. A total of two patients with allogeneic HSCT were alive and in CR. All patients without HSCT died of ANKL. The use of l-asparaginase was indicated as a factor for longer survival (HR 0.33, 95% confidence interval; 0.130.83, P = 0.02). Early diagnosis of ANKL, l-asparaginase-based chemotherapy and allogeneic HSCT might lead to improved patient outcomes.ArticleCANCER SCIENCE. 103(6):1079-1083 (2012)journal articl

Sinonasal Glomangiopericytoma Causing Oncogenic Osteomalacia

A 60-year-old woman suffered from recurrent femur neck fracture. Laboratory data showed serum hypophosphatemia, elevated alkaline phosphatase, normal serum calcium levels, and normal parathyroid hormone levels. Radiological examinations revealed a tumor in the right maxillary alveolar bone. The nasal cavity mass was removed, and the histological features were those of glomangiopericytoma. After removal of the tumor, some of the laboratory data normalized. Based on the clinical features, histopathological diagnosis and postoperative course of events, a diagnosis of glomangiopericytoma causing oncogenic osteomalacia was confirmed. We report a case of oncogenic osteomalacia caused by sinonasal glomangiopericytoma

Primary T-cell Lymphoma of the Thyroid Associated with Hashimoto's Thyroiditis, Histologically Mimicking MALT-Lymphoma

Most of thyroid lymphomas are B-lineage, and T-cell lymphomas are rare. Here, we report a case of primary thyroid T-cell lymphoma associated with Hashimoto's thyroiditis. A 48-yr-old woman presented with incidentally found neck mass. Histologically, the resected right lobe of the thyroid was replaced by monomorphic small atypical lymphoid cells with lymphoepithelial lesion-like change, most of which were immunoreactive for CD3, CD8, βF-1, and TIA-1. Peripheral T-cell lymphoma, unspecified, was finally diagnosed after molecular study for TCR-γ gene rearrangement. This is the second case of cytotoxic T-cell lymphoma reported in the thyroid gland so far. Unique association between thyroid follicles and neoplastic lymphocytes may be characteristic feature of this type of T-cell lymphoma

Bone Marrow is Involved in Less than 10% of Patients with Nasal-Type NK/T Cell Lymphoma at Initial Diagnosis

To evaluate the frequency of bone marrow involvement by nasal-type NK/T cell lymphoma, we retrospectively studied biopsy specimens from 40 patients by EBV in situ hybridization (ISH). Three patients had marrow involvement at initial diagnosis (7.5%). In one patient (1/40, 2.5%), the disease in bone marrow was recognized by routine morphological assessment, while two other patients had minimal involvement of lymphoma cells which was recognized only by EBV in situ hybridization (2/40, 5%). Two patients had a disseminated disease at diagnosis and died 6 days and 214 days after diagnosis. One patient had diffuse colonic lesion and died 82 days later. In conclusion, marrow involvement in nasal NK/T cell lymphoma is infrequent at initial diagnosis, and EBV ISH is a useful technique for identifying the minor subgroup of patients which have easily overlooked neoplastic involvement