Difference in Prognosis between T- and B-Cell Lymphomas: Clinical Study at Shikoku Cancer Center Hospital

In order to determine whether the surface marker phenotypesof non-Hodgkin's lymphomas affect the prognosis, we have studiedthe differences in response rate and duration of survival betweenT- and B-cell lymphomas. Sixty-four patients who underwent first-linetherapy, including combination chemotherapy and/or radiotherapy,from February 1979 to August 1985 were evaluated. With the aidof standard immunological methods and monoclonal antibodiesrelated to T-cells and B-cells. 21 T-cell lymphomas and 21 B-celllyrnphomas were identified. In the other 22 cases phenotypeswere not determined mainly because of the inability to obtainfresh samples. The complete remission rate was 100% for B-celllymphomas and 52.3% for T-cell lymphomas. The median survivaltime for patients with lymphomas of Stage III and IV. excludingthose with low-grade histology, was nine months for T-cell lymphomasand 17 months for B-cell lymphomas. T-cell lymphomas were foundto have significantly poorer prognosis than B-cell lymphomas.One patient with B-cell lymphoma and six patients in an undeterminedphenotype group, who were treated with combination chemotherapy,have been alive more than three years without relapse and thesepatients are considered potentially cured. Our results suggestthat the surface marker phenotype study of lymphoma cells aswell as histological subtying is important in prognosis andthat more effective therapy is needed to improve the prognosisof T-cell lymphomas.     

Combination chemotherapy with adriamycin, cyclophosphamide, vincristine, prednisolone (VEPA) in non-Hodgkin's lymphoma, with special reference to correlation of surface phenotype with response and survival

Thirty-seven previously untreated patients with advanced non-Hodgkin's lymphoma were treated with VEPA therapy. The complete remission (CR) rate was higher in the patients with diffuse B-cell lymphoma (75%) than in those with follicular B-cell lymphoma (20%) and T-cell lymphoma (42%). Two characteristics, i.e., elevated LDH and bone marrow involvement, were negatively associated with response rate in patients with diffuse lymphoma (B-, T-). The median duration of CR has not yet been reached, and the 2-year relapse-free rate was 64% for cases of diffuse B-cell lymphoma, while for T-cell lymphoma patients, the median duration of CR was 7 months. For diffuse B-cell lymphoma patients, the median survival has not yet been reached, and the 2-year survival rate was 57%. On the other hand, median survival for T-cell lymphoma patients was 12 months. VEPA therapy was less effective for the treatment of T-cell lymphoma, and a more intensive regimen should therefore be designed to overcome the potential aggressiveness of T-cell lymphoma.     

Treatment outcome and pattern of failure in 77 patients with sinonasal natural killer/T-cell or T-cell lymphoma

BACKGROUND: Sinonasal natural killer (NK)/T-cell or T-cell lymphoma behaves quite differently from other lymphomas. The objective of this study was to investigate clinical features, treatment outcomes, and failure patterns in patients with this type of sinonasal lymphoma. METHODS: From September, 1977 to December, 2000, 77 patients with sinonasal NK/T-cell lymphoma or T-cell lymphoma who had received radiotherapy (R/T), chemotherapy (C/T), or both (R/T and C/T) were evaluated retrospectively. RESULTS: Fifty-six patients (73%) had locoregional disease only, and 21 patients (27%) had systemic involvement. Forty-four patients (57%) achieved a complete remission (CR). The 5-year overall survival rate was 36% (median follow-up, 89 months). Achievement of CR was the only prognostic factor for survival in multivariate analysis. Among patients with locoregional disease, the CR rate was 63%, and the 5-year overall survival rate was 42%. Combined R/T and C/T or R/T alone resulted in better survival compared with C/T alone (5-year survival rates, 59%, 50%, and 15%, respectively; P = 0.01). Incidences of locoregional and systemic failure were 43% and 30%, respectively. Outcome was dismal for patients with systemic disease, with a CR rate of 43% and a 5-year survival rate of 25%. Only 2 of 21 patients had sustained remissions. The locoregional and systemic failure rates were 57% and 71%, respectively. CONCLUSIONS: Treatment outcomes were unsatisfactory for patients with locoregional and systemic sinonasal NK/T-cell or T-cell lymphoma. R/T could not control locoregional disease satisfactorily, and C/T was unable to eradicate systemic disease in many patients. High-dose therapy may be worth studying in these patients. New treatments should be investigated to increase remission rates, prevent failure, and improve survival.     

Primary non-Hodgkin's lymphoma of bone: a rare disorder with high frequency of T-cell phenotype in southern Taiwan

Primary non-Hodgkin's lymphoma of bone (PLB) is a rare disorder representing less than 1% of all non-Hodgkin's lymphomas and has rarely been reported in Taiwan. A retrospective clinicopathological study was performed according to the 2002 World Health Organization criteria and identified 14 cases during a 13-year period in 2 medical centers in southern Taiwan. There was male predominance (M:F = 6:1) with a median age of 42 and bone pain (6 patients, 43%) as the most common symptom. Half of the patients had monostotic and the other half polyostotic lesions. Axial skeletons (10 cases, 71%) were the most frequent sites of involvement. The staging results were stage I (9 patients, 64%), stage II (2, 14%) and stage IV (3, 21%). Eight cases (57%) were of B-cell phenotype and the remaining 6 (43%), T-cell. Histologically, 7 (50%) were diffuse large B-cell lymphomas (DLBCLs) and 5 (36%) anaplastic large cell lymphomas. Seven patients received chemotherapy and radiotherapy; 4 chemotherapy and 3 radiotherapy alone. Of the 11 patients with follow-up information, 6 (55%) died of disease within 1 year including 5 with T-cell lymphomas, while all the 5 patients surviving over 1 year were of B-cell phenotype. The overall 1-year survival rate was 45%. The survival of B-cell lymphomas was significantly better than T-cell tumors (p = 0.016, log-rank test). In summary, this study reported the largest series of PBL in Taiwan and confirmed that the majority was DLBCL and B-cell tumors had more favorable prognosis. As compared to the Western series, the cases showed a striking male predominance, higher percentage of axial skeleton involvement, higher relative frequency of T-lineage tumors and poorer prognosis.     

High-dose therapy and autologous stem cell transplantation in Korean patients with aggressive T/NK-cell lymphoma

The proportion of aggressive T/NK-cell lymphoma in Korea is larger than in the West, and it shows a lower response to conventional chemotherapy and poorer survival than diffuse large B-cell lymphoma. This study was undertaken to evaluate the response rate and survival and to document the prognostic factors in patients with T/NK-cell lymphoma who have undergone high-dose therapy (HDT). Eligibility for the study was a mature T/NK-cell lymphoma with initially poor risk (as high or high intermediate risk on age-adjusted International Prognostic Index) or relapsed cases. Twenty-eight patients from 6 centers were reviewed retrospectively. The M : F ratio was 20:8, and median age was 36 years (range 16--60 years). Twelve patients had unspecified peripheral T-cell lymphomas, 7 anaplastic large-cell lymphomas, 6 nasal T/NK-cell lymphomas, and 3 angioimmunoblastic T-cell lymphomas. Disease status at transplant were initially poor risk in 15, chemosensitive relapse in 8 and chemo-resistant relapse in 5 patients, respectively. A complete response (CR) after HDT comprised 20 patients, including 16 with continued CR. Absolute neutrophil count ( > 500/microl) recovered at a median 11 days after autologous stem cell transplantation in 26 patients. Two therapy-related mortalities occurred. Estimated 3-year event-free survival and overall survival (OS) (+/- SE) were 24+/- 9 and 42+/- 10 months, respectively. Only CR status after HDT influenced OS (P=0.000). Therefore, an initial approach with effective induction and HDT may result in a better outcome in T/NK-cell lymphoma.     

Clinical significance of immunophenotype in diffuse non-Hodgkin's lymphoma: with special emphasis on the clinical characteristics of T-cell lymphoma

The clinical significance of immunophenotyping of the non-Hodgkin's lymphomas (NHL) is still controversial. Therefore, we evaluated the prognostic significance of T- and B-cell phenotype in 102 patients having diffuse non-Hodgkin's lymphoma who had been treated in a majority with Adriamycin-containing regimens. The significant differences in pretreatment clinical variables between the two patient groups were the higher frequency of markedly elevated LDH (greater than or equal to X 2 normal) and the lower frequency of decreased cholesterol level in B-NHL (p less than 0.05). Patients with B-NHL had a higher complete remission (CR) rate (77% vs 59%: p = NS), a significantly better overall survival (62% vs 34% at 3-years: p less than 0.05) and longer duration of remission for all patients (50% vs 27% at 3-years: p less than 0.05). Among forty-five patients with stages III and IV disease of nodal origin (excluding those with diffuse small cleaved histology), the B-cell group was associated with a better prognosis; a higher CR rate (68% vs 41%: p = NS) and a longer duration of remission for all patients (44% vs 12% at 2-years: p less than 0.05). Furthermore, for patients with T-cell phenotype, the primary site was the only prognostic factor; the patients whose disease originated from Waldeyer's ring, nasal cavity, and paranasal sinuses, achieved a better CR rate (92% vs 40%: p less than 0.05) and a longer duration of remission for all patients (48% vs 15% at 2-years: p less than 0.05). We conclude that advanced T-cell lymphoma of nodal origin is a subgroup of patients with very poor prognosis if treated with less intensive chemotherapeutic regimens.     

Prognostic significance of the immunophenotype versus the International Prognostic Index in aggressive non-Hodgkin's lymphoma

The International Prognostic Index (IPI) is currently the most widely accepted prognostic factor system for patients with aggressive non-Hodgkin's lymphoma (NHL). However, in constructing the model, the immunophenotype of the disease was not used as an independent variable. The purpose of the present study was to assess and compare the prognostic significance of the immunophenotype (B-cell vs. T-cell) of aggressive NHL with other well-established prognostic determinants, in particular the IPI. Between January 1995 and December 2000, a retrospective analysis was conducted of clinical and pathological data on 181 patients aged = 15 years who had been newly diagnosed with aggressive NHL. All pathology slides were reviewed and defined according to the Revised European-American Lymphoma classification. Forty-one patients (23%) had T-cell lymphoma and 140 patients (77%) had B-cell lymphoma. Diffuse large B-cell lymphoma and unspecified peripheral T-cell lymphoma were the 2 most common entities, comprising 63% and 14% of patients, respectively. Most of the pretreatment characteristics, including IPI risk groups, were not significantly different between B-cell and T-cell lymphomas. The rates of complete remission (71% vs. 54%, P = 0.038) and progressive disease (39% vs. 63%, P = 0.023) significantly favored patients with B-cell lymphoma. With a median follow-up time of 31 months (range, 10-81 months), the 5-year overall survival (49% vs. 27%; P < 0.001) and event-free survival (35% vs. 10%; P < 0.001) were significantly better in B-cell lymphoma. The 5-year disease-free survival was also in favor of the B-cell group (48% vs. 21%; P = 0.086). Patients with T-cell lymphoma yielded inferior survival in all IPI risk groups. Multivariate analysis revealed T-cell lymphoma as the most significant factor associated with short overall survival (relative risk [RR], 3.4; 95% CI, 1.9-5.9) and event-free survival (RR 2.7, 95% CI, 1.7-4.3). When a second multivariate analysis was done using IPI (age, stage, performance status, number of extranodal sites, and serum lactate dehydrogenase) as one independent variable, T-cell phenotype remained the strongest factor affecting the survival of patients (P < 0.001). T-cell lymphoma is an independent prognostic factor, the significance of which is at least comparable to the IPI for patients with aggressive NHL.     

Comparison of peripheral T-cell lymphomas and diffuse large B-cell lymphoma

BACKGROUND: Peripheral T-cell lymphomas (PTCLs) are a biologically heterogeneous subgroup of lymphomas with poor prognosis. In this study, the authors analyzed the clinical behaviors of PTCLs and diffuse large B-cell lymphoma (DLBCL). METHODS: The authors compared the characteristics and outcomes of 59 patients with PTCLs, including 33 angioimmunoblastic T-cell lymphomas and 26 unspecified peripheral T-cell lymphomas, with the characteristics and outcomes of 193 patients with DLBCLs who were treated in the era before rituximab. RESULTS: Based on the clinical characteristics, elevated lactate dehydrogenase (LDH), poor PS, advanced stage, higher International Prognostic Index score, and B symptoms were more common in patients with PTCLs, and bulky mass was more common in patients with DLBCL. The rates of complete response (CR) or an unconfirmed CR (CRu) were higher in patients with DLBCL (72%) than in patients with PTCLs (56%; P = .03). The 5-year overall survival (OS), progression-free survival (PFS), and disease-free survival (DFS) rates were 31%, 26%, and 47%, respectively, in patients with PTCLs and 59%, 55%, and 73%, respectively, in patients with DLBCL (P = .001, P < .001, and P = .003, respectively). Although multivariate analysis identified several risk factors that were significant in PTCLs, but not in DLBCLs, for the CR/CRu, OS, PFS, and DFS rates, the immunophenotype was not identified as a risk factor. CONCLUSIONS: The poor response and survival of patients who had PTCLs, compared with patients who had DLBCL, was caused by numerous initial risk factors. T-cell phenotype itself did not appear to have a significant impact on either response or survival.     

High-dose chemotherapy with autologous hematopoietic stem cell transplantation for non-Hodgkin's lymphoma in complete response as consolidation therapy, second report

High-dose chemotherapy followed by autologous peripheral blood transplantation (HD-APBSCT) is a therapeutic option for patients with non-Hodgkin's lymphoma (NHL) after complete remission (CR) as consolidation therapy. In this report we describe a retrospective study of such treatment. A total of 38 patients with NHL were treated between November 19 9 1 and March 2005. At five years,the rate of disease-free survival (DFS) and overall survival (OS) was 64.3% and 66.5%, respectively. Patients who underwent transplantation in first CR had a 5-year probability of disease-free survival of 71.6% compared with 35.7% for those who were in second CR at the time of transplantation (p=0.10). In a monovariate analysis, second CR status at the time of transplantation was a relatively adverse predictor of DFS. None of those factors containing surface markers were significantly associated with clinical variables such as the CR status at the time of transplantation. Thirty high intermediate risk and high risk patients with aggressive B-cell lymphoma had a better outcome than patients treated with standard chemotherapy. In this study, 8 patients with T-cell lymphoma had a 3-year DFS and OS of 87.5% and 87.5%, respectively. HDT-APBSCT is a candidate for consolidation therapy for high-intermediate risk and high risk patients with aggressive B-cell and T-cell lymphoma.     

Long-term survival in Ki-1 lymphoma

Three patients with histologic and immunologic features of Ki-1-positive large cell lymphoma, who experienced long-term survival, are presented. These three patients at 2, 28, and 49 years of age had adenopathy; all cases had been initially misdiagnosed as metastatic carcinoma or malignant histiocytosis. On subsequent review, they had sinusal and diffuse growth of large pleomorphic cells that were Ber-H2 (Ki-1; CD 30) positive. One case marked as a T-cell lymphoma with UCHL1, one case expressed T-cell and B-cell markers, and one case was negative for both T-cell and B-cell markers. All patients received chemotherapy, and two received local radiation. One patient was not treated until 9 years after initial diagnosis. Two patients had several recurrences, but there has been no evidence of lymphoma in any of the three patients for 63 to 301 months; overall survival time has ranged from 14 to 25 years. These cases are the longest reported survivors with Ki-1 lymphoma; 5 years was the longest survival time previously reported. It also is noteworthy that Ber-H2 and other lymphoid-associated antigens appear to be preserved in formalin-fixed, paraffin-embedded tissues for prolonged periods. This may allow retrospective studies to evaluate the natural history of Ki-1 lymphomas, as well as their spontaneous or treatment-induced regression.     

Non-Hodgkin's lymphomas in a southern prefecture in Japan: an analysis in 715 cases

As of March 1978, the prognosis was confirmed in 715 non-Hodgkin's lymphoma cases reported during the 12-year period from 1965 through 1976 in the Prefecture of Kagoshima. Included were 222 cases of extranodal lymphoma. Patients with the initial tumor site in the gastrointestinal tract and Waldeyer's ring had a relatively good probability of survival. Among the patients with cutaneous lymphomas, those with the mycosis fungoides type had a poor survival rate. In Kagoshima Prefecture, the survival curve for cases of nodal lymphomas is characteristically much poorer than those reported in the medical literature. Of the nodal lymphomas, the nodular type was found in 128 cases and the five-year survival rate was 15%, while the diffuse type accounted for 365 cases and the five-year survival rate was 8%. The paracortex type, considered to be of T-cell origin, accounted for 55 cases and the five-year survival rate was 0%. These differences were statistically significant. Based on our data, the classification of nodular lymphomas into subtypes according to the degree of nodularity was found to be useful since it was related to clinical stage and survival. Rappaport's cellular classification of diffuse lymphoma is related to prognosis and was found to be useful, but problems still remain in determining T- and B-cell types by means of cellular morphology and thus further study is warranted.     

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.     

Peripheral T-Cell Lymphomas Respond Well to Vincristine, Adriamycin, Cyclophosphamide, Prednisone and Etoposide (VACPE) and Have a Similar Outcome as High-Grade B-Cell Lymphomas

Peripheral T-cell lymphomas (PTCL) represent a heterogeneous group of T-cell malignancies including subentities with favourable (large cell anaplastic) or unfavourable (pleomorphic) prognosis. The clinical outcome of PTCL has been controversially discussed, but a worse prognosis than high-grade B-cell Non-Hodgkin's lymphomas (NHL) has been postulated by most authors. In this report we summarize the results of a prospective comparative study investigating the therapy outcome of 27 patients (pts) with PTCL and 55 pts. with high grade B-cell NHL and give an overview of therapy studies in PTCL. The histological sub-types were 14 pleomorphic, 8 large-cell anaplastic (Ki-1+), 2 angioimmunoblastic (AILD) and 3 other PTCL. In three patients the PTCL was associated with non-tropical sprue (11%). Nineteen patients presented with an advanced stage of disease (stage III and IV, 70%), 17 (63%) pts. had B-symptoms. The patients were treated with vincristine 2 mg dl, adriamycin 25 mg/m² dl-3, cyclophosphamide 800 mg/m² dl, prednisone 60 mglm² dl-7 and etoposide 120 mg/m² dl-3 (VACPE). In 77% of pts. with PTCL and 84% of patients with high-grade B-cell NHL a complete remission (CR) was achieved. 75% of the complete responders with PTCL and 70% with B-NHL are still in ongoing CR. The subgroup of large-cell anaplastic attained a CR in 88%. The median observation time is 44 months (1+-77+). The probability of 1-, 3- and 5-year overall and disease-free survival for the T-cell group were 76%, 54%, 48% and 76%, 62%, 62%, respectively according to Kaplan-Meier. There was no significant difference regarding the remission rate, the overall-, event-free or disease-free survival compared to high-grade B-cell lymphomas.

In conclusion, the VACPE regimen is an effective and feasible regimen in the management of PTCL achieving complete remissions in a large proportion of patients.     

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.     

Phase II study of proteasome inhibitor bortezomib in relapsed or refractory B-cell non-Hodgkin's lymphoma.

PURPOSE: Evaluate efficacy and toxicity of bortezomib in patients with relapsed or refractory B-cell non-Hodgkin's lymphoma. PATIENTS AND METHODS: Patients were stratified, based on preclinical data, into arm A (mantle-cell lymphoma) or arm B (other B-cell lymphomas) without limitation in number of prior therapies. Bortezomib was administered as an intravenous push (1.5 mg/m2) on days 1, 4, 8, and 11 every 21 days for a maximum of six cycles. RESULTS: Sixty patients with a median number of prior therapies of 3.5 (range, one to 12 therapies) were enrolled; 33 patients were in arm A and 27 were in arm B, including 12 diffuse large B-cell lymphomas, five follicular lymphomas (FL), three transformed FLs, four small lymphocytic lymphomas (SLL), two Waldenstrom's macroglobulinemias (WM), and one marginal zone lymphoma. In arm A, 12 of 29 assessable patients responded (six complete responses [CR] and six partial responses [PR]) for an overall response rate (ORR) of 41% (95% CI, 24% to 61%), and a median time to progression not reached yet, with a median follow-up of 9.3 months (range, 1.7 to 24 months). In arm B, four of 21 assessable patients responded (one SLL patient had a CR, one FL patient had a CR unconfirmed, one diffuse large B-cell lymphoma patient had a PR, and one WM patient had a PR) for an ORR of 19% (95% CI, 5% to 42%). Grade 3 toxicity included thrombocytopenia (47%), gastrointestinal (20%), fatigue (13%), neutropenia (10%), and peripheral neuropathy (5%). Grade 4 toxicity occurred in nine patients (15%), and three deaths from progression of disease occurred within 30 days of withdrawal from study. CONCLUSION: Bortezomib showed promising activity in relapsed mantle-cell lymphoma and encouraging results in other B-cell lymphomas. Future studies will explore bortezomib in combination with other cytotoxic or biologic agents.     

Primary non-Hodgkin's lymphoma of the breast: retrospective analysis of prognosis and patterns of failure in two Australian centers

The breast is an uncommon site of presentation for primary non-Hodgkin's lymphoma, with prognosis and patterns of relapse still not clearly defined. A retrospective analysis of 21 patients presenting to 2 Australian centers during a 20-year period is presented. All patients were women and had a median age of 62 years. Fifteen patients (71%) had localized disease (12 unilateral and 3 bilateral), and 6 (29%) had regional lymph-node involvement. Histology was predominantly intermediate grade, with diffuse large B-cell lymphoma (DLBL) in 16 cases (76%). The most common treatment program was partial mastectomy followed by chemotherapy and radiation therapy (n = 12). Complete response (CR) to treatment was exhibited in 19 patients (90%), 11 of whom subsequently experienced relapse. Including the 2 patients who failed to exhibit an initial CR, the median time to disease progression was 23.4 months (range, 0-143 months), with a 5-year disease-free survival rate of 38% (+/- 12%). The actuarial median survival of all patients was 3.8 years, with bilateral breast involvement at presentation the only significant prognostic factor. The contralateral breast was the site of initial relapse in 3 patients (17%), all of whom subsequently died of disease. The actuarial rate of central nervous system (CNS) recurrence at 8 years was 39% (+/- 14%), occurring only in patients with diffuse large-cell histology. Our analysis suggests that DLBL presenting in the breast has a poor prognosis and characteristic patterns of failure. Targeted strategies such as CNS prophylaxis and contralateral breast irradiation might therefore improve prognosis and should be prospectively studied.     

Extranodal non-Hodgkin's lymphoma of the head and neck. A clinicopathologic study in the Kyoto-Nara area of Japan

The clinicopathologic features of 114 Japanese patients with extranodal non-Hodgkin's lymphoma of the head and neck region were analyzed. The median age was 60.5 years and the male:female ratio was 1.5:1. The most common site of involvement was Waldeyer's ring, followed by the oral cavity, thyroid gland, paranasal sinuses, nasal cavity, and larynx. Seventy-five percent of the patients were in Stage I or Stage II at admission. Histologically, diffuse lymphoma accounted for 94% and follicular lymphoma for 6% of cases. The histologic grade according to the Working Formulation System of the National Cancer Institute was low in 11%, intermediate in 75%, and high in 14% of cases. Immunohistochemical study showed that the majority of the cases were of B-cell type and only 13 cases (11%) were of the T-cell type. Peripheral T-cell lymphomas (eight cases) mainly occurred in the nasopharynx and nasal cavity, whereas four of five thymic T-cell lymphomas were found in the palatine tonsil. The over-all 5-year survival rate was 54%, and the factors affecting survival were sex, histologic grade, T/B phenotype, clinical stage, and the site of initial presentation. Five-year survival with nasal cavity and Waldeyer's ring lymphoma was 24% and 46%, respectively. The poor prognosis of lymphomas at these sites might result from the predominance of T-cell lymphoma, the paucity of low grade lymphoma, and the relatively high incidence of cases that were in an advanced stage at presentation. In Stage II, patients treated with combined therapy tended to have a better 5-year survival rate than those treated with radiotherapy alone.     

T-cell-rich B-cell lymphoma: a clinicopathologic study of 21 cases and comparison with 43 cases of diffuse large B-cell lymphoma

Clinicopathologic features of 21 patients with T-cell-rich B-cell lymphoma (TCRBCL) were reviewed and compared to 43 patients with diffuse large B-cell lymphoma (DLBCL) to determine if there were distinguishing clinical characteristics and differences in response or survival to CHOP therapy. For the diagnosis of TCRBCL, the current WHO criteria was used. In all of our cases, the majority of cells are non-neoplastic T cells and <10% large neoplastic B cells are present. The initial pathologic diagnosis was nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) in two cases. Patients with TCRBCL were significantly younger (median: 46 years) and had a significantly higher incidence of B symptoms (62%), hepatomegaly (33%) and marrow infiltration (33%) at presentation when compared to DLBCL (P<0.03). The CR rate after treatment was 48% for TCRBCL patients versus 79% for the DLBCL (P<0.003). Although the CR rates in between the two groups are significant, the difference in 3 years survival rates in each CR groups was insignificant (80% versus 77%). The overall survival time in the two groups was 17 months. Event-free survival time in TCRBCL was 12 months, compared with 17 months in the DLBCL (P>0.05). The frequency of patients with TCRBCL achieving CR was 52.6% whereas that of patients with DLBCL was 79% (P<0.003). The TCRBCL 3 years event-free survival 48% and overall survival 64% were 63 and 72% for DLBCL, respectively.     

Distribution and prognosis of WHO lymphoma subtypes in Taiwan reveals a low incidence of germinal-center derived tumors

To assess the distribution of lymphomas in Taiwan according to the WHO (World Health Organization) classification, 175 recently diagnosed cases of malignant lymphomas were studied and the clinicopathologic data were analyzed. B-cell lymphomas accounted for 57.1% of cases, T-cell lymphomas 38.9%, and Hodgkin's lymphoma 4%. Extranodal lymphomas predominated (55.4%). The most common subtype of B-cell lymphoma was diffuse large B-cell lymphoma (33.1%). All tumor types believed to be derived from germinal center (GC) B-cells including follicular lymphoma (4.6%), Burkitt lymphoma (1.7%), Hodgkin lymphoma (4.0%), and GC-like diffuse large B-cell lymphoma (as defined by combined expression of bcl-6 and CD10) were rather uncommon as compared to frequencies seen in series from Western countries. The common T-cell lymphomas included nasal and extranasal NK/T cell lymphoma (7.4%), mycosis fungoides (7.4%), and unspecified peripheral T-cell lymphoma (6.9%). Adult T-cell leukemia/lymphoma was very uncommon and accounts for only 0.6%. The proportional increase in T-cell lymphomas that were unrelated to type I human T-cell lymphotropic virus (HTLV-1) may be linked to differential Epstein-Barr virus (EBV) oncogenesis. The survival data revealed that mantle cell lymphoma, NK/T-cell lymphoma, unspecified peripheral T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma had an aggressive course. Our results confirm the utility of the WHO classification scheme for prognostic stratification and further highlight the distinctive distribution pattern of malignant lymphoma in Taiwan including the higher relative incidence of T cell lymphomas and the rarity of germinal center-derived B-cell tumors.     

14例原发性鼻窦非霍奇金淋巴瘤的临床分析

背景与目的:原发性鼻窦非霍奇金淋巴瘤(primary paranasal sinus lymphoma,PPSL)临床上较罕见,其生物学行为较独特.本文总结和分析PPSL的临床病理特点和治疗情况,以期增加对此病的认识.方法:回顾性分析1994～2006年中山大学肿瘤防治中心收治的14例PPSL,分析其临床和病理特点、治疗经过和随访结果.结果:14例中原发于上颌窦者11例,筛窦2例,蝶窦1例.根据淋巴瘤Ann Arbor分期,所有患者均为Ⅰ～Ⅱ期.根据美国癌症联合委员会(AJCC)的鼻窦实体瘤TNM分期标准,大部分患者(11/14)属于局部晚期T3～T4.病理类型:B细胞性12例(85.7%),其中弥漫大B细胞性占42.9%(6例);T细胞性1例,非B非T细胞性1例.2例接受上颌窦根治术,12例为肿物切除或活检;14例全部接受化疗,6例化疗后接受局部放疗.中位生存期59.5个月,全组5年总生存率和无事件生存率均为78.6%.结论:PPSL发病部位以上颌窦多见,病理类型以B细胞性为主,其中弥漫大B细胞性最常见,肿瘤局部侵犯广泛为其重要特征;采用全身化疗和局部放疗联合治疗,患者总体预后相对较好.