Expression of the natural killer (NK) cell antigen CD56 is uncommon in malignant lymphoma, but when it is, it is almost exclusively of the non-B cell lineage and show a preference for the nasal and nasopharyngeal region. T/NK cell lymphoma is known to be aggressive and refractory to treatment. It is highly associated with the Epstein-Barr Virus (EBV), but clinical investigations are rarely reported, that is until recently. We report here, on the clinical features and therapeutic outcomes of patients with T/NK cell lymphomas and its association with EBV. We reviewed fifty-four cases with peripheral T cell lymphomas in the upper aerodigestive tract between Jan. 1987 and Aug. 1998 from the Severance Hospital, Yonsei University College of Medicine. The diagnosis of T/NK cell lymphoma was made according to the expression of the NK cell markers, CD56 antigen and cytoplasmic CD3 epsilon, in tumor specimens, by immunohistochemistry. Epstein-Barr early region (EBER) RNA was detected using in situ hybridization on paraffin-embedded sections. Among the 54 cases with malignant lymphomas occurring in the upper aerodigestive tract, 20 had T/NK cell lymphoma (37%). The primary sites of T/NK cell lymphomas were the nasal cavity, 12 cases (60%), the tonsils, 4 cases (20%), the nasopharynx, 2 cases (10%), and the oropharynx, 2 case (10%). There were no differences between the features, at diagnosis or therapeutic modalities for patients with T/NK cell lymphoma and non-T/NK cell lymphoma. The complete remission rate of T/NK cell lymphomas was lower than non-T/NK cell lymphomas (65% vs 85%, p=0.02). The overall survival of T/NK cell lymphomas was 13 months (1-74 month), which was significantly lower than non-T/NK cell lymphomas [60.6% with a median follow up of 22 months (1-101 month, p=0.02)]. Disease free survival of T/NK cell lymphomas was 22 months (4-66 month), significantly lower than non-T/NK cell lymphomas [73.8% with a median follow up of 22 months (2-95 month), p=0.04]. The overall survival rates for T/NK cell lymphomas were significantly lower than for EBV positive non-T/NK cell lymphomas (p=0.018). EBER RNA was detected in the paraffin-embedded tissue sections of all T/NK cell lymphomas, compared to only 17.6% (6 of 34 cases) for non- T/NK cell lymphomas. In conclusion, as patients with T/NK cell lymphomas showed poor clinical outcomes, and a high association with EBV positivity, clinical trials with more investigational therapeutic strategies, and further research into the relationship of EBV infection with pathogenesis of T/NK cell lymphoma is warranted.
Adult ; Aged ; Digestive System Neoplasms/*therapy/virology ; Female ; Herpesvirus 4, Human/isolation & purification ; Human ; *Killer Cells, Natural ; Lymphoma/*therapy/virology ; Lymphoma, T-Cell/*therapy/virology ; Male ; Middle Age ; Respiratory Tract Neoplasms/*therapy/virology ; Treatment Outcome

Extranodal NK/T cell lymphoma is a relatively uncommon type of non-Hodgkin's lymphoma, which is prevalently distributed in Asia and South America, and is highly associated with Epstein-Barr virus (EBV) infection. Due to its highly aggressive course and poor response to treatment because of its multi-drug resistance, for the timebeing there is not yet a definite treatment strategy. The clinical manifestation, pathological diagnosis and the progress of treatment methods of ENTNKCL are reviewed below.
Epstein-Barr Virus Infections ; complications ; Herpesvirus 4, Human ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; virology

OBJECTIVETo evaluate the clinicopathological features and prognosis of primary hepatic lymphoma (PHL).

METHODSThirty-five patients with PHL who underwent surgical resection and were confirmed by pathology in our hospital from 1982 to 2012 were re-evaluated for clinicopathological data, including their symptoms, radiological features, recurrence interval, histopathological properties and prognosis.

RESULTSOf the 35 patients, 25 were men (71.4%) and 10 were women (28.6%), with an average age of 52.6 years old (range, 17-79 years). Presented symptoms were epigastric phymatosis, abdominal pain and low-grade fever. In the present study, 21 (60.0%) patients were positive for HBsAg, 1(2.9%) patient was positive for anti-HCV, 3 patients were positive for AFP, 12 patients and 2 patients were complicated by cirrhosis and hepatocellular carcinoma, respectively. Pathologically, 35 PHL were classified into 19 DLBCL (54.3%), 13 T cell-lymphoma (37.1%), and 3 MALT lymphoma (8.6%). Patients with DCBCL showed better postoperative survival than patients with T cell-lymphoma (31.7 ± 3.2) months vs. (22.9 ± 2.2) months (P < 0.05).

CONCLUSIONSHepatitis B virus (HBV) infection may contribute to the pathogenesis of Chinese patients with PHL. Surgical resection followed by comprehensive therapy is the first-line option for PHL. The prognosis of patients with PHL is associated with PHL subtypes.

Adolescent ; Adult ; Aged ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma, Hepatocellular ; pathology ; therapy ; virology ; Chemotherapy, Adjuvant ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Hepatitis B ; complications ; Hepatitis B Surface Antigens ; metabolism ; Hepatitis C Antibodies ; metabolism ; Humans ; Leukocyte Common Antigens ; metabolism ; Liver Cirrhosis ; complications ; Liver Neoplasms ; pathology ; therapy ; virology ; Lymphoma ; pathology ; therapy ; virology ; Lymphoma, B-Cell, Marginal Zone ; pathology ; therapy ; virology ; Lymphoma, Large B-Cell, Diffuse ; pathology ; therapy ; virology ; Lymphoma, T-Cell ; pathology ; therapy ; virology ; Male ; Middle Aged ; Prednisone ; therapeutic use ; Retrospective Studies ; Survival Rate ; Vincristine ; therapeutic use ; Young Adult ; alpha-Fetoproteins ; metabolism

No abstract available.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Biopsy ; Chemoradiotherapy, Adjuvant ; Diagnostic Errors ; Dupuytren Contracture/diagnosis ; *Fingers/pathology/ultrasonography/virology ; Hematopoietic Stem Cell Transplantation ; Herpesvirus 4, Human/genetics ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; *Lymphoma, Extranodal NK-T-Cell/diagnosis/therapy/virology ; Male ; Middle Aged ; Neoadjuvant Therapy ; Predictive Value of Tests ; RNA, Viral/genetics ; *Tendons/chemistry/pathology/ultrasonography/virology ; Treatment Outcome ; Tumor Markers, Biological/analysis ; Ultrasonography, Doppler, Color

OBJECTIVETo study the clinicopathologic features and prognostic factors of extranodal nasal type NK/T-cell lymphoma (EN-NK/TCL) in Chinese patients.

METHODSFifty-five cases of EN-NK/TCL diagnosed in Chinese patients during the period from 1998 to 2007 were studied by light microscopy, immunohistochemistry and in-situ hybridization. The follow-up information was analyzed.

RESULTSThe male-to-female ratio was 1.89:1. The median age of the patients was 38 years. The commonest sites of involvement included nasal cavity and adjoining tissue (85.5%). Histologically, EN-NK/TCL was composed of small to medium-sized lymphoid cells. Angiocentric and angiodestructive growth patterns, coagulative tumor necrosis and apoptotic bodies were frequently observed. Immunohistochemical study showed that CD20, the B-cell marker, was negative in all cases. The positivity rates for T-cell markers CD3epsilon, CD4, CD5 and CD8 were 100% (49/49), 7% (3/46), 8% (4/48) and 63% (29/46), respectively. Most cases were also positive for NK-cell marker CD56 (79% 42/53). All cases expressed cytotoxic granule-associated proteins TIA-1 and granzyme B. Only 17% (8/46) of the cases were positive for anti-apoptotic protein bcl-2. The proliferation index, as demonstrated by Ki-67 immunostain, varied: 30% (14/47) with a low Ki-67 expression level (< or = 29%), 28% (13/47) with a medium level (30%-59%) and 42% with a high level (> or = 60%). There was a significant positive correlation between the bcl-2 positive expression and a high Ki-67 expression level. In-situ hybridization for EBV-encoded RNA was positive in all cases. Amongst the 41 cases with clinical information available, 63.4% presented with Ann Arbor stage I to II. The performance status score was 1 in 87.8% cases. High lactate dehydrogenase level was demonstrated in some patients (31.8%). Amongst the 27 cases with follow-up data available, the median survival was 13 months. The overall 1-year, 2-year and 5-year survival rates were 52%, 31% and 20%, respectively. In general, cases with high proliferation index carried poor prognosis.

CONCLUSIONSEN-NK/TCL is a mature T-cell and NK-cell neoplasm which can be accurately diagnosed by histologic examination, immunohistochemical study and in-situ hybridization. The prognosis is usually not favorable. Proliferation index of the tumor represents an independent prognostic factor.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; CD3 Complex ; metabolism ; CD56 Antigen ; metabolism ; Child ; Epstein-Barr Virus Infections ; virology ; Female ; Follow-Up Studies ; Granzymes ; metabolism ; Herpesvirus 4, Human ; Humans ; Immunophenotyping ; Ki-67 Antigen ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; therapy ; virology ; Male ; Middle Aged ; Neoplasm Staging ; Nose Neoplasms ; metabolism ; pathology ; therapy ; virology ; Poly(A)-Binding Proteins ; metabolism ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; RNA, Viral ; analysis ; Retrospective Studies ; Survival Rate ; T-Cell Intracellular Antigen-1 ; Young Adult

Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Epstein-Barr Virus Infections ; Female ; Herpesvirus 4, Human ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; drug therapy ; pathology ; Neoplasms, Second Primary ; drug therapy ; pathology ; Prednisone ; therapeutic use ; Vincristine ; therapeutic use

The clinicopathologic features of a Korean patient with adult T-cell leukemia/lymphoma(ATLL) are presented. A 51-year-old man, who has lived in Korea since birth, had multiple cutaneous nodules and multiple lymphadenopathy for the previous two months. A histopathologic study of the lymph node and skin lesion revealed T-cell non-Hodgkin's lymphoma of pleomorphic type, medium and large cell type. Peripheral blood examination showed leukemic features with 30% of abnormal lymphoid cells. HTLV-I proviral DNA pX region was detected in the DNA from peripheral blood mononuclear cells(PBMC) and the specific gag, pol, and env HTLV-I sequences were detected in the lymph node using polymerase chain reaction technique. Human T-cell leukemia/lymphoma type I(HTLV-I) antibodies were present in the serum. An immunophenotypic study of the lymph node revealed CD4 positive and CD8 negative helper/inducer T cell type surface markers. This case is the acute type, i.e. prototypic ATLL. He was treated with an intensive chemotherapy including cyclophosphamide, etoposide, doxorubicin, vincristine, and prednisone. Despite initial transient improvement, the tumor progressed after three cycles of the regimen and became refractory to further chemotherapy. These clinicopathologic findings, including the immunophenotypic analysis, established with certainty the diagnosis of HTLV-I-induced adult T-cell leukemia/lymphoma.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Case Report ; Cyclophosphamide/administration & dosage ; DNA, Viral/blood ; Doxorubicin/administration & dosage ; Etoposide/administration & dosage ; Human ; Human T-lymphotropic virus 1/isolation & purification ; Immunophenotyping ; Korea/epidemiology ; Leukemia-Lymphoma, T-Cell, Acute, HTLV-I-Associated/drug therapy/epidemiology/pathology/virology ; Lymph Nodes/pathology ; Male ; Middle Age ; Prednisone/administration & dosage ; Proviruses/isolation & purification ; Tumor Stem Cells/chemistry/pathology ; Vincristine/administration & dosage