Malignant lymphoma in southern Taiwan according to the revised European-American classification of lymphoid neoplasms

BACKGROUND: The purpose of the current study was to determine the distribution and relative frequency of each subtype of malignant lymphoma in southern Taiwan according to the revised European-American classification of lymphoid neoplasms (REAL). METHODS: The pathology files of a regional hospital in southern Taiwan for 1989-1998 were searched for malignant lymphoma, lymphoproliferative disorder, and Hodgkin disease (HD). The results of light microscopy, immunohistochemistry, and in situ hybridization for Epstein-Barr virus-encoded RNA (EBER) were correlated with clinical findings, and all cases were classified according to REAL. RESULTS: A total of 205 cases were analyzed retrospectively. There were 197 cases (96.1%) of non-Hodgkin lymphoma (NHL) and 8 cases (3. 9%) of HD. Among the 197 NHL cases, 161 (81.7%) were of B-cell lineage and 36 (18.3%) were of T-/natural killer cell lineage. Diffuse large B-cell lymphoma, extranodal marginal zone lymphoma, and follicular lymphoma were the most common B-cell subtypes and represented 47.2%, 19.3%, and 6.1%, respectively, of all NHL cases. Among the 36 cases of T-/natural killer cell lineage, unspecified peripheral T-cell lymphoma (8.6%), T-/natural killer cell lymphoma (angiocentric lymphoma) (4.1%), and anaplastic large cell lymphoma (3.6%) were the most common subtypes. Seven of eight T-/natural killer cell lymphoma cases were positive for EBER. The eight cases of HD were classified as lymphocyte-rich classic (two cases), nodular sclerosis (two cases), and mixed cellularity (four cases) subtypes. Three of these eight cases were positive for EBER. CONCLUSIONS: To the authors' knowledge this study is the first in Taiwan using the REAL classification and it again confirms the different geographic distribution of the various subtypes of malignant lymphoma. The frequency of T-/natural killer cell lineage NHL in Taiwan is higher than that in Western countries but not as high as reported previously.     

Non-Hodgkin lymphoma in Chile: a review of 207 consecutive adult cases by a panel of five expert hematopathologists

The distribution of subtypes of non-Hodgkin lymphoma (NHL) in Latin America is not well known. This Chilean study included 207 consecutive cases of NHL diagnosed at five cancer centers in the capital, Santiago, and one center in Vi?a del Mar. All cases were reviewed and classified independently by five expert hematopathologists according to the 2001 World Health Organization classification of NHL. A consensus diagnosis of NHL was reached in 195 of the 207 cases (94%). B-cell lymphomas constituted 88% of NHL, and diffuse large B-cell lymphoma (DLBCL, 38.5%) and follicular lymphoma (25.1%) were the most common subtypes. There was a high frequency of marginal zone B-cell lymphoma (10.3%), as well as of extranodal natural killer (NK)/T-cell lymphoma, nasal type (2.6%) and adult T-cell leukemia/lymphoma (0.5%). Extranodal presentation was seen in 74 of the 195 cases (38%) and the most common extranodal presentation was in the stomach (37.6%). The most common gastric lymphoma was DLBCL (54.5%) followed by mucosa-associated lymphoid tissue (MALT) lymphoma (41%). Overall, the frequency of NHL subtypes in Chile is between that reported in Western and Eastern countries, which is probably a reflection of the admixture of ethnicities as well as the environment and socioeconomic status of its population.     

Malignant lymphomas of bone in Japan

Thirty-four cases of primary non-Hodgkin's lymphoma (NHL) of bone collected from hospitals in Japan were histologically and immunohistologically reviewed, and the results were compared with those in Western countries. There were no remarkable differences in age, sex, and the distribution of bone tumors between Japanese and Western cases. Sixteen cases (47%) diagnosed previously as "reticulum cell sarcoma" were reclassified as diffuse NHL of large cell (seven cases), mixed type (four cases), immunoblastic type (three cases), clear cell type (one case), and multilobated type (one case). The cases with small lymphocytic type with plasmacytoid features (lymphoplasmacytic type) were more common in Japan (35% of our cases) than in Western countries. The distribution of histologic subtypes, except for lymphoplasmacytic and T-cell lymphomas, in the current cases was similar to that in Western countries. However, immunohistochemistry showed that Japanese cases contained a much higher frequency of T-cell lymphoma (10% of all cases) than Western cases. Histologic grade according to the Working Formulation correlated well with clinical stage. There may be a tendency towards better prognosis with lower grade tumors, but this was not statistically significant.     

Chromosomal abnormalities of 200 Chinese patients with non-Hodgkin's lymphoma in Taiwan: with special reference to T-cell lymphoma.

BACKGROUND: The distribution of the histopathological subtypes of non-Hodgkin's lymphoma (NHL) is different among various geographical areas. However, there are few reports concerning cytogenetic findings of NHL, especially T-cell lymphoma, in Asian people. PATIENTS AND METHODS: We analyzed the chromosomal abnormalities of 200 adult patients with NHL in Taiwan and correlated the non-random aberrations with the histological subtypes. RESULTS: One hundred and thirty-eight patients (69%) had B-cell lymphoma. The incidence of the t(14;18) in total lymphoma was lower in Taiwan (12%) than in the West (20-30%), but its incidence in follicular lymphoma was comparable between the two areas (17 of 28 patients, 61% versus approximately 50-60%). Sixty-two patients (31%) had T-cell lymphoma, including 11 angiocentric T/natural killer (NK)-cell lymphoma and only two angioimmunoblastic T-cell lymphoma (AILD). The recurrent chromosomal abnormalities in T-cell lymphoma comprised 6q deletion (30%), 11q deletion (20%), 17p deletion (16%), -17 (16%), -Y (14%) and + 8 (11%). Angiocentric T/NK-cell lymphoma had a significantly higher frequency of 1q duplication (P=0.001), 6p duplication (P <0.001) and 11q deletion (P=0.011) than other T-cell lymphoma. The incidences of +3 and +5, two common abnormalities in AILD, were quite low in T-cell lymphoma in Taiwan (4% and 2%, respectively), compared with those in the West (16-32% and approximately 15%, respectively). The 11q deletion, not a common aberration in T-cell lymphoma in western countries, occurred quite frequently in Taiwan. CONCLUSIONS: The chromosomal aberrations of NHL are quite different among various geographical areas, which may reflect the differences in the distribution of the histological subtypes of lymphoma among various areas.     

A Collaborative Nationwide Lymphoma Study in Lebanon: Incidence of Various Subtypes and Analysis of Associations with Viruses

Incidence of various Hodgkin (HL) and non-Hodgkin lymphoma (NHL) subtypes and association with viruses in Lebanon are not known. We undertook a nationwide study of 272 patients diagnosed with lymphoma in 2007. HL comprised 32.7 % (n?=?89) of cases while NHL represented 67.3 % (n?=?183). Consistent with the literature, nodular sclerosis was the most predominant HL subtype (n?=?57/89). Among NHL, B-cell NHL represented 88 % (n?=?161/183), T-cell NHL 9 % (n?=?17/183), whereas in 2.7 % it was not classifiable. The B-cell NHL comprised predominantly diffuse large B-cell lymphoma (46 %) and follicular lymphoma (23 %). 81 cases were reviewed by a panel of pathologists with 87.6 % concordance rate. Serology was negative for hepatitis C in 122 tested cases. HIV was positive in 2 cases. Two adult T-cell leukemia/lymphoma were HTLV-I positive. EBV IgG were positive in 88.5 % of cases. 38 EBV seropositive cases [27 NHL (24 B-cell, 3 T-cell) and 11 HL] were studied for EBV genome expression using EBV-encoded RNA (EBER)-in situ hybridization. EBER expression was positive in 8 (21 %) cases (6 HL, 2 T-cell NHL). The distribution of lymphoma subtypes in Lebanon appears similar to that of Western countries. The high rate of EBV positivity in HL and T-cell lymphoma by EBER deserves further investigation.     

Splenic lymphoproliferative disorders in human T lymphotropic virus type-I endemic area of japan: clinicopathological, immunohistochemical and genetic analysis of 27 cases

Primary splenic involvement in lymphoid neoplasms is rare and the clinicopathologic features of splenic lymphoma are not well described compared to nodal non-Hodgkin's lymphoma (NHL). Here we characterized splenic lymphomas in an human T lymphotropic virus type-I (HTLV-I) endemic area of Japan. To assess the pattern of splenic involvement and evaluate prognosis, we reviewed 27 specimens consisting of 26 splenectomies and one necropsy, which were classified using REAL classification. Cases were divided into primary splenic lymphoma in 11 patients and secondary in 16 patients. The incidence of primary splenic lymphoma was 0.3% (11 of approximately 4,000 malignant lymphomas). Primary splenic lymphomas included 7 diffuse large B cell lymphoma (DLBL), 2 follicular lymphomas (FL), and 1 each of splenic marginal zone lymphoma (SMZL) and anaplastic large cell lymphoma (ALCL). Secondary splenic lymphomas included 6 DLBL, 4 mantle cell lymphoma (MCL), 2 FL, 2 Hodgkin's disease (HD), 1 each of hairy cell leukemia and ALCL. Gross examination showed two patterns of splenic involvement; solid type (formation of large nodular mass, n=16) and disseminated type (multiple nodules with diffuse infiltration but no large nodular formation, n=10). The type could not be determined in one case. Most solid types were DLBL or FL, while MCL was of the disseminated type. Immunohistochemistry showed all but each 2 cases of ALCL and HD were of B lineage. Follow-up of 26 patients indicated that all but one patient with primary lymphoma were still alive (range, 1-89 months) and 8 of 15 patients with secondary lymphomas died due to the progression of malignant lymphoma; the survival rate at 2 years was 50% in these patients. No elevation of anti-HTLV-I antibody was found. In situ hybridization for Epstein-Barr virus (EBV) showed no reactivity of lymphoma cells, although a few small lymphocytes were positive for EBV. Hepatitis C virus was observed in 6 of 20 (30%) patients examined and 4 of 11 (36%) cases of primary splenic lymphoma. Our findings indicate that patients with primary splenic lymphoma have a favorable prognosis after splenectomy.     

Splenic Lymphoproliferative Disorders in Human T Lymphotropic Virus Type-I Endemic Area of Japan: Clinicopathological, Immunohistochemical and Genetic Analysis of 27 Cases

Primary splenic involvement in lymphoid neoplasms is rare and the clinicopathologic features of splenic lymphoma are not well described compared to nodal non-Hodgkin's lymphoma (NHL). Here we characterized splenic lymphomas in an human T lymphotropic virus type-I (HTLV-I) endemic area of Japan. To assess the pattern of splenic involvement and evaluate prognosis, we reviewed 27 specimens consisting of 26 splenectomies and one necropsy, which were classified using REAL classification. Cases were divided into primary splenic lymphoma in 11 patients and secondary in 16 patients. The incidence of primary splenic lymphoma was 0.3% (11 of approximately 4,000 malignant lymphomas). Primary splenic lymphomas included 7 diffuse large B cell lymphoma (DLBL), 2 follicular lymphomas (FL), and 1 each of splenic marginal zone lymphoma (SMZL) and anaplastic large cell lymphoma (ALCL). Secondary splenic lymphomas included 6 DLBL, 4 mantle cell lymphoma (MCL), 2 FL, 2 Hodgkin's disease (HD), 1 each of hairy cell leukemia and ALCL. Gross examination showed two patterns of splenic involvement; solid type (formation of large nodular mass, n = 16) and disseminated type (multiple nodules with diffuse infiltration but no large nodular formation, n = 10). The type could not be determined in one case. Most solid types were DLBL or FL, while MCL was of the disseminated type. Immunohistochemistry showed all but each 2 cases of ALCL and HD were of B lineage. Follow-up of 26 patients indicated that all but one patient with primary lymphoma were still alive (range, 1-89 months) and 8 of 15 patients with secondary lymphomas died due to the progression of malignant lymphoma; the survival rate at 2 years was 50% in these patients. No elevation of anti-HTLV-I antibody was found. In situ hybridization for Epstein-Barr virus (EBV) showed no reactivity of lymphoma cells, although a few small lymphocytes were positive for EBV. Hepatitis C virus was observed in 6 of 20 (30%) patients examined and 4 of 11 (36%) cases of primary splenic lymphoma. Our findings indicate that patients with primary splenic lymphoma have a favorable prognosis after splenectomy.     

Proteasome inhibition with bortezomib: a new therapeutic strategy for non-Hodgkin's lymphoma

The incidence of non-Hodgkin's lymphoma (NHL) has markedly increased in the US and other westernized countries in recent years and presents a considerable clinical challenge. NHL is divided into subtypes that follow an aggressive or indolent course. Follicular lymphoma (FL), the most common indolent subtype, and mantle cell lymphoma (MCL), an aggressive subtype that accounts for approximately 5% of cases, are generally incurable. MCL has a relatively poor prognosis, with a median survival of 3-4 years. Despite improving response rates with new agents and regimens, the lack of demonstrated improvement in overall survival in many subtypes supports the development of novel approaches, such as proteasome inhibition. Bortezomib is the first proteasome inhibitor to be evaluated in human studies. It has already been approved as second-line treatment in multiple myeloma and is now under active investigation in NHL. The US FDA has granted bortezomib fast-track designation for relapsed and refractory MCL. In vitro and in vivo studies have demonstrated single-agent activity against various lymphoid tumors, and additive or synergistic effects in combination with other agents, including standard chemotherapy drugs employed in NHL. Phase 2 clinical trials indicate that bortezomib is well tolerated and active in several NHL subtypes, with response rates of 18-60% in FL and 39-56% in MCL. A number of combination trials are currently underway with a range of standard agents. Bortezomib has the potential to play a significant role throughout the NHL treatment algorithm in the future.     

SOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas

Mature T- and NK-cell neoplasms (MTNKN) collectively represent a rare disorder, representing less than 15% of all non-Hodgkin lymphoma (NHL) cases and qualifying for orphan disease designation by the U.S. Food and Drug Administration (FDA). These consist of 9 families in the fifth revised WHO classification of lymphoid neoplasms, which are made up of over 30 disease subtypes, underscoring the heterogeneity of clinical features, molecular biology, and genetics across this disease group. Moreover, the 5 most common subtypes (peripheral T-cell lymphoma, not otherwise specified; nodal TFH cell lymphoma, angioimmunoblastic type; extranodal NK-cell/T-cell lymphoma; adult T-cell leukemia/lymphoma; and ALK-positive or -negative anaplastic large cell lymphoma) comprise over 75% of MTNKN cases, so other subtypes are exceedingly rare in the context of all NHL diagnoses and consequently often lack consensus on best practices in diagnosis and management. In this review, we discuss the following entities–enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and primary cutaneous ɣδ T-cell lymphoma (PCGD-TCL) – with an emphasis on clinical and diagnostic features and options for management.     

Quantitative analysis detects ubiquitous expression of apoptotic regulators in B cell non-Hodgkin's lymphomas.

To determine whether the expression levels of Bcl-2 family apoptotic regulators are correlated with the histopathological heterogeneity of B cell non-Hodgkin's lymphomas (NHL), we quantified their expression in malignant B cell populations isolated from 33 biopsy samples, including small lymphocytic lymphoma (SLL, n = 9), mantle cell lymphoma (MCL, n = 8), follicular lymphoma (FL, n = 8), and diffuse large cell lymphoma (DLCL, n = 8). Normal B cells purified from reactive lymph nodes and tonsil (n = 3) were used as controls. Cell lysates were analyzed by Western blotting, and signals quantified by densitometry. Expression of Bcl-2 and its homologues, Bcl-xL, Bcl-xS, Bax, Bad, Bak and Bag-1, was detected in all NHL cases, with wide variations between histological subtypes and within each subtype. Statistically significant differences were: (1) a higher level of Bad expression in DLCL compared to FL and MCL; (2) a lower level of Bak expression in FL compared to DLCL, SLL and MCL; and (3) a higher Bag-1 expression level in FL compared to SLL. When compared to NHL cells, normal B cells showed a higher level of Bax expression, and a lower level of Bcl-xL expression. Thus, quantitative analysis shows ubiquitous expression of Bcl-2 family proteins in normal and neoplastic B cells; the variations in expression levels may contribute to both the B-NHL clinicopathological diversity and the different apoptotic sensitivities of normal B cells vs B-NHL cells.     

Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas

We analyzed 108 cases of non-CLL non-Hodgkin lymphoma (NHL) associated with autoimmune hemolytic anemia (AIHA) (+/- pure red cell aplasia (PRCA)) or Evans' syndrome. The analysis was based on cases reported in the literature, which were retrieved by means of Pubmed and Medline searches and of an original series of 121 patients with NHL as well as reference lists of papers in the field. The number of cases in various NHL subtypes was small (n = 6 - 25). Nevertheless, interesting and sometimes unexpected differences in sex prevalence, temporal relationship between onset of lymphoma and AIHA, stage of lymphoma, relative frequency of warm antibody-AIHA (WA-AIHA) and cold antibody (CA-AIHA), association with PRCA and response of AIHA to treatments were noted for various lymphoma entities. WA-AIHA was more frequent in B-cell lymphomas, while CA-AIHA and PRCA predominantly occurred in T-cell lymphomas. Anti-lymphoma treatment seemed to be more effective against AIHA than conventional therapy with steroids or immunoglobulin. Although generated by a literature survey, this compilation of data indicates a complex relation of lymphoma and AIHA and warrants more attention and specific studies.     

Practical Utility of the Revised European-American Classification of Lymphoid Neoplasms for Japanese Non-Hodgkin's Lymphomas

A clinicopathological study of 515 non-Hodgkin's lymphoma (NHL) cases was performed using the revised European-American classification of lymphoid neoplasms (REAL classification) in an HTLV1-nonendemic area of Japan. The following characteristics were revealed: 1) frequency of extranodal lymphomas was high (59%) with 79% B-cell lymphomas in this series, while the overall ratio of B:T/NK lineage was 3.7:1; 2) the most common type was the diffuse large B-cell lymphoma (46%), follicle center lymphomas occurred at an incidence lower (15%) than that in European and American populations, and marginal zone B-cell lymphomas accounted for as much as 12%; 3) peripheral T-cell lymphomas were common (19%), with the unspecified type predominant (11%), while adult T-cell lymphomas were present at a level equivalent to that among European and American patients (1%). Clear segregation of survival curves was rated according to cell lineage and B-cell lymphomas had a better prognosis than T/NK-cell lymphomas. Furthermore, new subtypes in the REAL classification, such as marginal zone B-cell and mantle cell lymphomas, exhibited distinct curves. Taken altogether, the REAL classification demonstrated advantages for assessment of Japanese NHL cases.     

Practical utility of the revised European-American classification of lymphoid neoplasms for Japanese non-Hodgkin's lymphomas.

A clinicopathological study of 515 non-Hodgkin's lymphoma (NHL) cases was performed using the revised European-American classification of lymphoid neoplasms (REAL classification) in an HTLV1-nonendemic area of Japan. The following characteristics were revealed: 1) frequency of extranodal lymphomas was high (59%) with 79% B-cell lymphomas in this series, while the overall ratio of B:T/NK lineage was 3.7:1; 2) the most common type was the diffuse large B-cell lymphoma (46%), follicle center lymphomas occurred at an incidence lower (15%) than that in European and American populations, and marginal zone B-cell lymphomas accounted for as much as 12%; 3) peripheral T-cell lymphomas were common (19%), with the unspecified type predominant (11%), while adult T-cell lymphomas were present at a level equivalent to that among European and American patients (1%). Clear segregation of survival curves was rated according to cell lineage and B-cell lymphomas had a better prognosis than T / NK-cell lymphomas. Furthermore, new subtypes in the REAL classification, such as marginal zone B-cell and mantle cell lymphomas, exhibited distinct curves. Taken altogether, the REAL classification demonstrated advantages for assessment of Japanese NHL cases.     

Use of the World Health Organization (WHO) classification of non-Hodgkin's lymphoma in Mumbai, India: a review of 200 consecutive cases by a panel of five expert hematopathologists

This study aims to answer the question whether the World Health Organization (WHO) classification of non-Hodgkin's lymphoma (NHL) can be practised to international standards at the Lymphoma Registry (LR) established at the Tata Memorial Hospital, Mumbai, India. Furthermore, the study aims to identify differences in the distribution of NHL subtypes at this LR (likely to be representative of India) as compared to the rest of the world. A panel of 5 expert hematopathologists from the NHL Classification Project reviewed 200 consecutive NHL cases at the LR in January of 2001. These cases were accrued during August and September, 2000. On all cases, hematoxylin and eosin stains and appropriate immunostains were available for review. The diagnosis made by the host pathologist at the LR (KNN) and the initial diagnosis made by each of the expert hematopathologists was compared with the consensus diagnosis. A consensus diagnosis was made by the 5 experts in 197 cases. The agreement of the host pathologist with the consensus diagnosis was 82% and the agreement of the individual experts with the consensus diagnosis varied from 76-88% (mean 82%). According to the consensus diagnosis, 80% of NHLs were of B-cell type, 18% were of T-cell type, and the immunophenotype could not be determined in the remaining 2% of cases. In conclusion, the WHO classification of NHL was properly utilized at the Lymphoma Registry, Mumbai, India, and geographic differences were noted in the distribution of NHL subtypes at the LR as compared to the rest of the world. Precursor T lymphoblastic leukemia/lymphoma was more common in India (7%) than the rest of the world (1-4%), and indolent B-cell NHLs (29%) were less common than in the West. As compared to China and Japan, peripheral T-cell lymphoma (4.6%), extranodal NK/T cell lymphoma, nasal type (0.5%) and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma) (2.6%) were less common, but follicular lymphoma (15%) and chronic lymphocytic leukemia/small lymphocytic lymphoma (5%) were more common. This suggests that the distribution of the B-cell and T-cell lymphomas in the Indian population, except for lymphoblastic lymphoma, lies in between the Western world (mainly Caucasian) and the Orientals.     

Somatic Hypermutations in the VH Segment of Immunoglobulin Genes of CDS-positive Diffuse Large B-Cell Lymphomas

De now CDS-positive (CD5+) diffuse large B-cell lymphoma (DLBL) has recently been identified as constituting a homogeneous subgroup with distinct clinicopathologic and genotypic characteristics, but its origin remains to he elucidated. Previous studies by sequence analysis of the variable region of the immunoglobulin heavy chain (V_H) have shown that CDS⁵ B-cell malignancies such as mantle cell lymphoma (MCL) and B-cell chronic lymphocytic leukemia (B-CLL) cells represent pre-geminal center (pre-GC) stage B cells in contrast with the post-GC stage of most DLBLs, which show somatic hyper mutations in V_H genes. In the present study, we investigated the V_H sequence of de novo CD5⁺ DLBL to clarify whether CD5⁺ DLBL represents the pre-GC stage, as do other CD5⁺ B-cell malignancies, or the post-GC stage, as is typical of DLBL. All eight cases (four CDS⁴ DLBL and four CDS-negative (CD5⁻) DLBL) examined by us showed somatic hypermutatiohs in the V_H segment and two of the CD5⁻ DLBL cases showed intra-clonal diversity, suggesting that CD5⁺ DLBLs were derived from the same maturation stage as CD5⁻ DLBL, but were distinct from the other indolent CD5⁺ B-cell lymphomas of B-CLL and MCL. These data suggest that de novo CD5⁺ DLBLs do not merely lie within a continuous spectrum with B-CLL and MCL, but represent a biologically distinct variant within the diagnostic framework of diffuse large B-cell lymphoma.     

Clinical characteristics and outcome for hepatitis C virus-positive diffuse large B-cell lymphoma

Several previous studies have addressed the association between hepatitis C virus (HCV) infection and non-Hodgkin lymphoma (NHL), but there are few studies on HCV-related diffuse large B-cell lymphoma (DLBL). We conducted this retrospective study to investigate the distinctive clinical characteristics and outcome for HCV-positive DLBL. We compared the clinical characteristics and outcomes of 32 HCV-positive DLBL cases from nine Korean institutions with those of 371 HCV-negative DLBL cases. A higher percentage of HCV-positive DLBL cases were associated with old age (≥60) than HCV-negative DLBL cases at diagnosis (59.4% vs. 36.1%, respectively, P = 0.009) and HCV-positive cases were less likely than HCV-negative cases to have extranodal involvement (53.1% vs. 71.1%, respectively, P = 0.044). The nodal presentation was the only independent factor favorably influencing the event free survival (EFS) in HCV-positive DLBL (HR = 0.11, 95% CI; 0.01 - 0.95, P = 0.012). In comparison to patients with HCV-negative DLBL, HCV-positive DLBL patients had a superior complete response rate (P = 0.023) and EFS (P = 0.02). In Korean patients, HCV-positive DLBL is more common with old age and has less extranodal involvement than does HCV-negative DLBL. The superior survival outcome for HCV-positive DLBL should be verified by further investigation, especially with respect to its correlation with transformed low-grade NHL.     

112例化疗有效的非霍奇金淋巴瘤的临床病理特征及预后分析

目的：研究化疗有效的非霍奇金淋巴瘤（NHL）的临床病理特征,探讨其预后和治疗。方法：回顾性分析112例经初治或经复治获得完全缓解的NHL的临床病理特点、化疗方式以及随访结果。结果：（1）112例NHL中,B系占76.8%（86/112）,以弥漫大B细胞淋巴瘤（DLBCL）最常见,占40.2%（45/112）;NK/T系占23.2%（26/112）,以非特殊性外周T细胞性淋巴瘤（PTCL）最常见,占12.5%（14/112）。（2）复治病例获得完全缓解时平均化疗周期数为9个,显著多于初治病例（平均化疗周期数为4个）（P〈0.05）。（3）套细胞性淋巴瘤（MCL）和B淋巴母细胞性淋巴瘤（B-LBL）复治例数比例分别为57.1%（4/7）和66.7%（2/3）,获得CR时化疗周期数均为12个,显著多于其他组织类型（P〈0.05）。（4）本组112例患者均随访3年以上,其中82例随访5年以上,3年和5年生存率分别为50.0%（56/112）和35.4%（29/82）。生存率主要与年龄、B症状、临床分期、LDH水平以及复发情况有关（P〈0.05）,而与性别和发生部位无关（P〉0.05）。结论：NHL具有明显的异质性,应根据患者各自的临床病理特征及组织学类型综合考虑治疗计划,制定适宜的化疗方案。     

Treatment of T-cell non-hodgkin’ lymphoma

Opinion statement T-cell non-Hodgkin’s lymphoma (NHL) represents approximately 10% to 15% of all lympho-mas in Western countries. Patients with T-cell NHL are often treated similarly to patients with intermediate grade B-cell NHL, although many reports have demonstrated lower overall survival rates in patients with T-cell NHL compared to patients with B-cell NHL. Updated classifications have recognized specific clinical and pathologic T-cell entities, such as peripheral T-cell lymphoma, not otherwise characterized, angioimmunoblastic lymphoma, systemic anaplastic T-cell lymphoma, adult T-cell leukemia/lymphoma, subcutaneous panniculitis-like T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal natural killer (NK)/T-cell lymphoma nasal type, and enteropathy-type intestinal T-cell lymphoma. Furthermore, these distinct T-cell NHL subtypes often warrant individual-ized diagnostic and therapeutic strategies, such as the associated cytophagic histiocytic panniculitis and hemophagocytic syndrome with subcutaneous panniculitis-like T-cell lymphoma, the chromosomal translocation t(2;5), leading to the nucleophosmin anaplastic lymphoma kinase fusion protein, viral pathogenesis of Epstein-Barr virus, human T-cell lymphotropic virus type-1 associated with extranodal NK/T-cell lymphoma nasal type and adult T-cell leukemia/lymphoma, respectively, and the role of radiation therapy in extra-nodal NK/T-cell lymphoma nasal type. Other active therapeutic agents in T-cell NHL include purine and pyrimidine antimetabolite agents eg, nucleoside analogues and gemcitabine, respectively), denileukin diftitox, and antinucleoside or retinoic acid with interferon-α combination treatment. The exact role of transplantation in patients with T-cell NHL is unknown, but several case series have documented the feasibility of autologous and allogeneic transplant with reported long-term survival rates similar to transplanted B-cell NHL. Identification of relevant proto-oncogenes and tumor suppressor genes involved in the pathogenesis of T-cell NHL, such as the nucleophosmin anaplastic lymphoma kinase fusion protein, p53 and retinoblastoma gene, cyclin-dependent kinase inhibitors, histone deacetylation inhibitors, and infectious etiologies (eg, Epstein-Barr virus and Helicobacter pylori), in addition to their interplay with the various regulatory pathways of cell-cycle progression and apoptosis, represent potential candidates for molecular-based therapy. Prospective multi-institution clinical trials are critically important to determine the most effective treatment regimens that will continue to improve cure rates in these aggressive, yet treatable and often curable, diseases.     

Epidemiology of the non-Hodgkin's lymphomas: Distributions of the major subtypes differ by geographic locations

Background: There has been no previous systematic study of the distribution of the major subtypes of non-Hodgkin's lymphoma (NHL) across geographic regions, although there have been isolated reports of such differences.Design: As part of a clinical evaluation of the International Lymphoma Study Group (ILSG) classification of NHL, we classified 1378 NHLs from eight different geographic sites (Omaha, NE, USA; Vancouver, BC, Canada; Capetown, South Africa; London, England; Würzburg/Göttingen, Germany; Lyon, France; Locarno/Bellinzona, Switzerland; and Hong Kong) using the ILSG classification.Results: Substantial differences were found in the distribution of the major subtypes of NHL across geographic regions (P < 0.0001). A greater percentage of follicular lymphoma was seen in North America, London and Capetown (31% versus 14% at other sites). Peripheral T-cell lymphoma was more common in London, Capetown and Hong Kong (9%) than elsewhere (3%). In Locarno/Bellinzona, higher percentages of mediastinal large B-cell lymphoma (9% versus 2% elsewhere) and mantle cell lymphoma (14% versus 6% elsewhere) were seen. Angiocentric nasal T-/NK-cell lymphoma was only seen in Hong Kong (8%) and Lyon (2%).Conclusions: Our study provides evidence that the distribution of NHL subtypes differs by geographic region. These findings suggest that geographical differences in etiologic or host factors may be responsible for the observed differences in the distribution of cases across NHL subtypes.