To explore the clinical and pathological characteristics of hepatosplenic gammadelta T-cell lymphoma and its relationship with Epstein-Barr virus infection, the clinical features of a 9-year-old girl with hepatosplenic gammadelta T-cell lymphoma were investigated, the smears of bone marrow was stained with Wright' s stain, biopsies of bone marrow and liver specimen were embedded in plastic and sliced about 4 microm in thickness and routinely stained with HE staining, the immunohistochemical staining was used to mark the tumor cells, and EBER probes were used to detect Epstein-Barr virus RNA. The results showed that the girl presented with prolonged fever, anemia, thrombocytopenia, hepatosplenomegaly, chronic active Epstein-Barr virus infection, and elevated levels of serum ferritin and lactate dehydrogenase. Bone marrow aspirate revealed the infiltration of atypical lymphocytes in the bone marrow stroma. The liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids, which was positive for the T-cell associated marker CD3 and activated cytotoxicity-associated marker granzyme B. In-situ hybridization analysis with EBER probes revealed that the above-mentioned characteristics were negative in neoplastic cells. It is concluded that hepatosplenic gammadelta T-cell lymphoma is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Hepatic and/or splenic and/or bone marrow biopsy with combined phenotype is beneficial to diagnosis. Epstein-Barr virus infection is late event involving an already transformed gammadelta T-cell clone.
Child ; Epstein-Barr Virus Infections ; complications ; Female ; Humans ; Liver Neoplasms ; diagnosis ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; diagnosis ; pathology ; virology ; Receptors, Antigen, T-Cell, gamma-delta ; analysis ; Splenic Neoplasms ; diagnosis ; pathology ; virology

OBJECTIVETo study the clinicopathologic features of various types of mature T-cell and natural killer (NK)/T-cell lymphoma in Guangdong, China, with respect to the 2008 WHO classification of lymphoid neoplasms.

METHODSEleven hundred and thirty-seven (1137) cases of mature T-cell or NK/T-cell lymphoma diagnosed during the period from 2002 to 2006 in Guangzhou area were retrieved. The clinical data, histologic features and immunohistochemical findings were reviewed by a panel of experienced hematopathologists. Additional immunostaining was performed if indicated. The cases were re-classified according to the 2008 WHO classification of lymphoid neoplasms.

RESULTSNine hundred and sixty-three (963) cases fulfilled the diagnostic criteria of mature T-cell or NK/T-cell lymphoma and accounted for 20.1% of all cases of lymphoma encountered during the same period (963/4801). A predominance of extranodal involvement was noted in 644 cases (66.9%), while 319 cases (33.1%) showed mainly nodal disease. The prevalence of various lymphoma subtypes was as follows: peripheral T-cell lymphoma, unspecified (PTCL, NOS) 293 cases (30.4%), extranodal NK/T-cell lymphoma, nasal type 281 cases (29.2%), anaplastic large cell lymphoma (ALCL) 198 cases (20.6%), and angioimmunoblastic T-cell lymphoma (AILT) 46 cases (4.8%). The male-to-female ratio was 1.99. The median age of the patients was 44 years, with the peak age of PTCL, NOS, extranodal NK/T-cell lymphoma, nasal type and AILT being 55 to 64 years, 25 to 54 years and 65 to 74 years, respectively. ALK-positive ALCL occurred more frequently in young age, while the ALK-negative ALCL cases occurred mainly in the elderly.

CONCLUSIONSExtranodal lesions predominate in mature T-cell and NK/T-cell lymphomas occurring in Guangzhou area. There is a male predominance and the overall incidence shows no increasing trend with age of the patient. The peak age of various subtypes however varies. The most common subtype was PTCL, NOS, followed by extranodal NK/T-cell lymphoma, nasal type, ALCL and AILT. The relatively frequent occurrence of extranodal NK/T-cell lymphoma, nasal type in Guangdong area is likely associated with the high incidence of Epstein-Barr virus infection there.

Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; China ; Epstein-Barr Virus Infections ; Female ; Humans ; Immunoblastic Lymphadenopathy ; metabolism ; pathology ; virology ; Infant ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; virology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; virology ; Lymphoma, T-Cell ; classification ; metabolism ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; virology ; Male ; Middle Aged ; Protein-Tyrosine Kinases ; metabolism ; Receptor Protein-Tyrosine Kinases ; Retrospective Studies ; Sex Factors ; World Health Organization ; Young Adult

OBJECTIVETo study the clinical features, immunophenotypes and the significance of Epstein-Barr virus infection in primary nasal and pharyngeal non-Hodgkin's lymphomas in Shenyang.

METHODSOne hundred and fifty eight cases of primary nasal and pharyngeal non-Hodgkin's lymphomas were included in this study. The samples were stained with haematoxylin and eosin for histological examination. Immunohistochemistry studies were performed using monoclonal antibodies, including CD3 for T-lymphocytes, CD20 for B-lymphocytes, and CD56 and CD57 for NK cells. All cases were reclassified according to the new WHO classification of lymphomas (2001). In situ hybridization detection of EBV-encoded small nuclear RNA (EBER-1) was performed in 99 cases.

RESULTSOverall, 101 (63.9%) of the 158 NHL were extranodal NK/T cell lymphomas (nasal type), 23 (14.6%) were nonspecific peripheral T cell lymphomas and the remaining 34 cases (21.5%) were B cell lymphomas. The primary sites of involvement were the nasal cavity (53.2%, 84/158), the tonsil (24.7%, 39/158) and the pharynx (22.1%, 35/158). Among 99 cases studied by EBER-1 in situ hybridization, a positive detection was seen in 70/71 cases (98.6%) of extranodal NK/T cell lymphoma (nasal type), 8/12 cases (66.7%) of T cell lymphoma, and 7/16 cases (43.8%) of B cell lymphoma.

CONCLUSIONSAmong primary nasal and pharyngeal NK lymphomas, extranodal NK/T cell lymphoma (nasal type) is the most common type and is strongly associated with EBV infection. The pathological diagnosis of nasal and pharyngeal lymphomas should take considerations of the anatomic sites and immunophenotypical features.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; CD3 Complex ; metabolism ; CD56 Antigen ; metabolism ; Child ; Female ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Lymphoma, B-Cell ; metabolism ; pathology ; virology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; virology ; Lymphoma, Non-Hodgkin ; metabolism ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; virology ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; metabolism ; pathology ; virology ; Pharyngeal Neoplasms ; metabolism ; pathology ; virology ; RNA, Viral ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; virology ; Young Adult

The incidence of posttransplantation lymphoproliferative disorders (PTLDs) has increased in recent years. Although rare, various types of T-cell lymphoma have been reported and their association with Epstein-Barr virus (EBV) has been compared with B-cell PTLDs. We report a case of splenic peripheral T-cell lymphoma occurring in a 47-yr-old male patient 7 yr after renal allograft transplantation. The spleen showed sinusoidal proliferation of focal CD30 positive, large, atypical lymphoid cells. Positivity for CD3 and cytolytic granule-associated proteins was also demonstrated in the tumor cells, while anaplastic large cell lymphoma kinase (ALK) and CD8 were not expressed. Strong nuclear signals for EBV mRNA were noted by EBER1 in situ hybridization. A molecular genetic study demonstrated a rearrangement of the gamma T-cell receptor gene. To our knowledge, this case is unique in terms of a posttransplant T-cell lymphoma that shows focal CD30, cytolytic granule-associated proteins, and EBV positivity.
Antigens, CD30/genetics ; Antigens, CD30/metabolism ; Herpesvirus 4, Human/genetics ; Herpesvirus 4, Human/metabolism* ; Human ; Kidney Transplantation* ; Lymphoma, T-Cell, Peripheral/pathology ; Lymphoma, T-Cell, Peripheral/virology* ; Male ; Membrane Proteins/metabolism ; Middle Aged ; RNA, Viral ; RNA-Binding Proteins/metabolism ; Serine Endopeptidases/metabolism ; Splenic Neoplasms/pathology ; Splenic Neoplasms/virology*

OBJECTIVETo investigate the expression and possible role of C-type lectin-like natural killer cell receptors, including CD94 and NKG2s, in extranodal NK/T-cell lymphoma, nasal type (EN-NK/T-NT).

METHODSReverse transcriptase polymerase chain reaction (RT-PCR) was used to detect the expression of CD94 and NKG2s in tissue sections of 21 cases of EN-NK/T-NT(confirmed by histology, immunohistochemistry, in-situ hybridization for Epstein-Barr virus(EBV) and PCR for T-cell receptor genes), eight midline B cell lymphomas (BCL), 10 peripheral T cell lymphoma of lymph nodes (PTCL), five spleens, five thymuses and five chronic nasopharyngitis.

RESULTSAll 21 cases of EN-NK/T-NT showed typical histological features, with expression of CD3epsilon, CD56, cytotoxic granules and positivity of EBV in 20 cases. The RT-PCR results showed a high level expression of CD94 (85.7%) and NKG2 members (95.2% totally, with NKG2A/2B in 85.7%, NKG2D in 61.9%, NKG2F in 14.3%, NKG2C/2E in 4.8%, respectively and sequentially) in EN-NK/T-NT. But in the controls, none of the receptors were detected in TCL (0%) and BCL (0%), while only a few cases of lymphoid tissues expressed one or two of these receptors (two spleens and two chronic nasopharyngitis mucosa for CD94, one spleen for NKG2A/2B and one thymus for NKG2D). The differences of CD94 and NKG2 expression between EN-NK/T-NT and BCL or TCL were statistically significant (P<0.01). Co-expression of CD94 and NKG2 was found in 17 out of 21 EN-NK/T-NT cases (81.0%).

CONCLUSIONSThe specific and sequential expression nature of CD94 and NKG2 in EN-NK/T-NT, mimics the developmental expression model in their normal counterparts, and suggests that the tumor cells of most cases are being activated and keeping in a stage as the functional NK cells. Detection of these molecules may provide a useful tool to confirm the diagnosis of NK cell lymphoma.

Adolescent ; Adult ; Aged ; Diagnosis, Differential ; Epstein-Barr Virus Infections ; virology ; Female ; Follow-Up Studies ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Lymphoma, B-Cell ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; metabolism ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; NK Cell Lectin-Like Receptor Subfamily C ; metabolism ; NK Cell Lectin-Like Receptor Subfamily D ; metabolism ; Nose Neoplasms ; diagnosis ; metabolism ; pathology ; virology ; Survival Rate ; Young Adult

OBJECTIVETo compare the detection rate of Epstein-Barr virus latent membrane protein 1 (LMP1) 30 base pair deletion in extranodal nasal type NK/T-cell lymphoma with that in chronic inflammation of nasopharynx and tonsillitis; and to analyze the prognostic significance of LMP1 deletion in extranodal nasal type NK/T-cell lymphoma.

METHODSPolymerase chain reaction was used to detect the deletion of LMP1 in 55 cases of extranodal nasal type NK/T-cell lymphoma and 19 cases of chronic inflammation of nasopharynx and tonsillitis. Follow-up information of 1 to 58-month duration was available in 33 patients.

RESULTSIn all the 55 extranodal nasal type NK/T-cell lymphoma cases studied, 9 cases contained the wide-type or predominantly wide-type LMP1. On the other hand, 46 cases contained the deleted or predominantly deleted LMP1. In the non-lymphoma control group, 16 cases contained the deleted or predominantly deleted LMP1. However, no statistically significant difference was found in the detection rate of 30 base pair deleted LMP1 between extranodal nasal type NK/T-cell lymphoma and control group (P > 0.05). The prognosis of deleted or predominantly deleted LMP1 in extranodal nasal type NK/T-cell lymphoma was worse.

CONCLUSIONThough 30 base pair deletion of Epstein-Barr virus LMP1 may not be an important pathogenetic step in extranodal nasal type NK/T-cell lymphoma, it may play some role in tumor progression.

Adolescent ; Adult ; Aged ; Base Sequence ; Epstein-Barr Virus Infections ; genetics ; virology ; Female ; Follow-Up Studies ; Herpesvirus 4, Human ; genetics ; Humans ; Killer Cells, Natural ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; genetics ; virology ; Male ; Middle Aged ; Nasopharyngitis ; Nose Neoplasms ; genetics ; virology ; Sequence Deletion ; Survival Rate ; Tonsillitis ; genetics ; virology ; Viral Matrix Proteins ; genetics ; isolation & purification

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