scholarly journals RT-DeVIC Therapy Is Effective for Localized NK/T Cell Lymphoma Patiens

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1702-1702 ◽  
Author(s):  
Kyoko Ueda ◽  
Noriko Nishimura ◽  
Yuko Mishima ◽  
Hideaki Nitta ◽  
Yoshiharu Kusano ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
T Cell ◽  
Local Control ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Free Survival ◽  
Nk T Cell ◽  
Grade 3

Abstract BACKGROUNDS: Extranodal natural killer (NK) /T cell lymphoma, nasal type is much common in East Asia than in Western countries. CHOP therapy is not effective for NK/T cell lymphoma because of the drug resistance induced by P glycoprotein. Yamaguchi et al reported the effectiveness of concurrent radiotherapy and DeVIC (RT-DeVIC) therapy for localized nasal NK/T cell lymphoma. Nowadays, RT-DeVIC therapy is recognized as a standard treatment. So far, we have limited information about this treatment because NK/T cell lymphoma is rare phenotype. PATIENTS AND METHODS: We reviewed retrospectively the patients with localized NK/T cell lymphoma treated with RT-DeVIC therapy. Radiation therapy was administered for a total dose of 50 Gy. Concurrently, chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) was performed up to 3 cycles. The primary objectives of this analysis were to evaluate the response rates and progression free survival (PFS) and overall survival (OS). RESULTS: A total of 20 patients who diagnosed as nasal NK/T cell lymphoma between April 2007 and October 2012 were analyzed. Sixteen patients were stage 1E and 4 were Stage 2E. As the NK/T cell lymphoma prognostic index, 6 patients were group 1, 10 were group 2, 3 were group 3, and 1 was group 4. Seventeen patients completed 3 cycles of DeVIC therapy and 19 patients completed planned radiation therapy. Overall response rate (ORR) was 75% and CR rate was 70% in the entire patients. Local control was 90%. Half of the patients who reached CR showed long time survival without disease progression. On the other hand, 7 of 14 patients relapsed after CR, and all 5 patients experienced systemic failure. The sites of relapse were paranasal sinuses (n=2), skin (n=3), brain (n=1), testis (n=1). Among them, one patient reached 2nd CR. However, 5 patients were not eligible for salvage chemotherapy, because lymphoma progressed rapidly and their general condition became worse. Six patients did not reach CR after RT-DeVIC therapy. Five of them experienced systemic relapse and median survival of them was only 8 months. The median follow up time was 17.6 months (range 2 – 77.9 months). Median overall survival was not reached and median progression free survival was 14.6 months. Risk factors predicted of OS or PFS were not clear. All entire patients experienced grade 3 or 4 neutropenia. Mucositis was common non-hematological toxicity and it was the major cause of grade 3 or 4 appetite loss. Only one patient discontinued RT-DeVIC due to grade 3 mucositis, grade 3 dermatitis and septic shock. CONCLUSION: We reviewed treatment outcomes of 20 cases of RT-DeVIC therapy. In this analysis, the majority of relapsed or refractory cases showed systemic disease and the prognosis of these patients were poor. However, RT-DeVIC therapy showed excellent local control and response rates which were similar to the prior study. The effectiveness of RT-DeVIC therapy for patients with NK/T cell lymphoma was reconfirmed. Disclosures No relevant conflicts of interest to declare.

Nodal Peripheral T-Cell Lymphoma – a Clinical and Epidemiological Analysis at Medicine School of Sao Paulo University

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1706-1706
Author(s):  
Luis Alberto de Padua Covas Lage ◽  
Marianne Castro Goncalves ◽  
Rodrigo Santucci ◽  
Renata Oliveira Costa ◽  
Debora Levy ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
High Risk ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Free Survival ◽  
Large Cell Lymphoma

Abstract Background: Peripheral T-cell lymphoma (PTCL) are a biologically and clinically heterogeneous group of rare diseases arising from mature or activated post-thymic T lymphocytes. Correspond to 10% to 15% of lymphoid malignancies with marked geographical variation in incidence. According to the WHO classification they are divided into nodal, extranodal, primary cutaneous and leukemic or disseminated and encompass 18 distinct entities. The nodal group involves the peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), angioimmunoblastic lymphoma (AITL), anaplastic large cell lymphoma ALK positive (ALCL-ALK+) and anaplastic large cell lymphoma ALK negative (ALCL-ALK-). The literature of PTCL is scarce, especially in our country where data of epidemiology, clinical features and outcomes are usually rarely available. So, to better understand PTCL we performed a retrospective study with patients treated in a reference service for cancer treatment in Brazil. Methods: Eight-seven nodal PTCL patients treated with anthracyclne-based regimen (CHOP or, CHOEP) from January 2000 to June 2014 were evaluated retrospectively at the Medicine School of Sao Paulo University, Brazil. All patients lower than 60 years were consolidated with autologous hematopoietic stem cell transplantation (ASCT) in first CR or PR except that with ALCL-ALK+ diagnosis. Refractory and relapsed patients were salvaged with 3-4 cycles of IVAC (Ifosphamide 1.5 g/m2 i.v D1-D5, etoposide 100mg/m2 i.v D1-D5, aracytin 2g/m2 i.v twice a day D1-D2) regimen and submitted to ASCT. It was performed a central histopathological review and clinical and epidemiological data were obtained from medical records. Patients were evaluated for overall response (OR) including complete response (CR) and partial response (PR), overall survival (OS) and progression free survival (PFS). Statistical analysis was performed using the STATA-3 program using and a p-value ≤ 0.05 was considered statistically significant. Results: Of the 87 patients, 34 (39.08%) cases were classified as ALCL-ALK-, 27 (31.03%) as PTCL-NOS, 16 (18.39%) as ALCL-ALK+, 6 (6.89%) as AITL and in 4 (4.1%) cases the diagnosis could not be performed and an expansion of the immunohistochemical is ongoing. Thirty-six (45.38%) cases were female and 51(54.62%) were male, 59(67.81%) patients were lower than 60 years. Seventy-six (87.35%) patients presented in advanced stage (III or IV) at diagnosis but 73(83.90%) patients presented an ECOG < 2 and 14(16.10%) ≥ 2. Eighteen (20.70%) patients were of low-risk, 26 (29.88%) of low-intermediate risk and 43(49.42%) of high-intermediate and high-risk of international prognostic index (IPI). The CR and PR was obtained for 44(50.57%) and 8(9.19%), respectively with 59.76% OR. Thirty (34.48%) patients were primary refractory and five remain under treatment. In a median of follow of 30 months, ALCL-ALK+ show higher OS (median 140.98 months) than ALCL-ALK- (44.20 months), PTCL-NOS (median 20.62 months) and AITL (median 7.24 months) (p=0.41) (Figure 1A). The median of PFS was 3.84 months for AITL, 23.44 months for ALCL-ALK+, 40.03 months for PTCL-NOS and was not yet reached for ALCL-ALK- (p=0.0006) (Figure 1B). Figure 1: Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 1:. Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 2 Figure 2. Conclusion: In this study we showed that ALCL-ALK+ as well as found in the literature presented a better OS in comparison to others nodal T-cell lymphoma as AITL, PTCL-NOS and ALCL-ALK-. Surprisingly the PFS of ALCL-ALK+ was statistically significant lower than of ALCL-ALK-. We thought that this result may be explained because in our service until to perform this analysis we did not indicate ASCT in first CR for ALCL-ALK+, but for all ALCL-ALK-. This hypothesis may be reinforced as the most of our cases presented high-intermediate and high-risk of IPI and that could equalize the favorable effect of ALK expression. In addition, we changed our approach and we are also indicating ASCT in first line for patients with ALCL-ALK+ with intermediate-high and high-risk of IPI . Disclosures No relevant conflicts of interest to declare.

scholarly journals Outcome of Autologous Hematopoietic Cell Transplantation in First Complete Remission(CR1) in Patients with Peripheral T-Cell Lymphomas(PTCLs)

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3315-3315
Author(s):  
Shuo Liu ◽  
Zhengming Jin ◽  
Depei Wu ◽  
Haiwen Huang
Keyword(s):  
Overall Survival ◽  
Stem Cell ◽  
T Cell ◽  
Cell Transplantation ◽  
Cumulative Incidence ◽  
Cell Lymphoma ◽  
Statistical Significance ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Free Survival

Abstract Background Patients with peripheral T cell lymphomas (PTCLs) generally have a poor prognosis with conventional chemotherapy. Most studies demonstrates that, compared to the patients who did not achieve complete remission (CR) after initial therapy, the patients of PTCL who received autologous stem cell lymphoma (ASCT) as consolidation treatment show clearly advantage in survival. However, given the absence of randomized controlled studies, it is unproven that clinical value of consolidative ASCT for PTCL patients achieving CR1. There is a possibility that the survival is similar with or without up-front ASCT group. Thus, we collected the data of PTCL patients who attain CR1 following conventional chemotherapy in our center during the past 10 years. And the objective of this study is to evaluate overall survival(OS), progression-free survival(PFS), and cumulative incidence of relapse (CIR) between observation and first-line ASCT group. Patients and Methods Weconducted a retrospective study of patients with PTCL who were treated in our center from January 2009 to April 2019. The histopathologic diagnosis of all PTCL patients according to the World Health Organization classification. Exclusion criteria were the following: (1) anaplastic lymphoma kinase (ALK)-positive anaplastic large T-cell lymphoma; (2) cutaneous T cell lymphoma (CTCL); (3) concurrent B cell lymphomas; (4) natural killer/T-cell lymphoma (NK/TCL); (5) patients who underwent allogeneic stem cell transplantation. Furthermore, patients with PTCL age ≤65 years were included. Overall survival(OS )and progression-free survival(PFS) rates were estimated using the Kaplan-Meier method and Survival was compared using the log-rank test. Cumulative incidence of relapse (CIR) was compared by Gray's test competing risk test statistic. The level of statistical significance was set at p < 0.05. Results A total of 97 patients who met inclusion criteria were enrolled in our center from January 2009 to April 2019. And 59 (59/97, 60.8%)achieved CR1 after receiving induction chemotherapy. Table 1 summarizes the baseline characteristics for the patients in CR1. Of the 59 patients, 43 patients underwent observation and waiting in CR1, 16 patients underwent consolidative ASCT. PTCL NOS accounted for more than 50% at diagnosis in both groups. However, there was significant difference in median age between Non-ASCT group and ASCT group. Patients receiving ASCT were younger and in better physical condition. There were no difference in initial chemotherapy between two groups. Median follow-up time in the entire patient cohort for CR1 (59) was 31months. The median OS and PFS for patients who underwent observation in CR1 was 105 months and 20 months, the median OS and PFS for patients who underwent ASCT as consolidation treatment was 133 months and 91 months. There were no statistical significance in OS (105m vs. 133m, P=0.541) (Figure 1) and PFS (20m vs. 91m, P=0.237) (Figure 2). The estimated 2-year OS was 68.7% and 74.5% in the non-ASCT group and ASCT group, respectively. The estimated 2-year PFS was 41.9% and 62.5%, respectively. When considering incidence of disease relapse, the 2-year cumulative incidence of relapse in the non-ASCT and ASCT group was 41% and 25%, respectively. Again, however, this did not meet statistically significant(P=0.504) (Figure 3). Notably, among patients with advanced-stage disease, elevated LDH, extranodal involvement>1 sites or intermediate-to-high IPI scores, patients who received ASCT as consolidative treatment did not have long time survival compared to the non-ASCT group. Conclusion In conclusion, for PTCL patients achieving CR1 following induction therapy, consolidative ASCT does not extend overall survival and progression-free survival compared to observation. Similarly, consolidative ASCT also failed to reduce cumulative incidence of relapse. We favor proceeding to observe and wait because of high toxic of hematopoietic stem cell transplantation. However, The finding still needs to be confirmed in a larger, prospective study. Table 1 Disclosures No relevant conflicts of interest to declare.

Clinical Outcomes of Patients with T Cell NHL Undergoing Allogeneic Stem Cell Transplant.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3026-3026 ◽  
Author(s):  
Jasmine Zain ◽  
J. Palmer ◽  
N. Tsai ◽  
L. Popplewell ◽  
A. Nadamanee ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
B Cell ◽  
Sample Size ◽  
Clinical Outcomes ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Cell Transplant ◽  
Free Survival

Abstract Background: T cell NHL represent approximately 10–15% of all lymphomas. Pts with T cell NHL are often treated similarly to patient with B cell NHL, although the clinical outcomes of most patients with T- NHL tend to be worse with the exception of ALK positive anaplastic large cell lymphomas (ALCL). Updated classifications in recent years have recognized specific clinical and pathologic T cell entities with distinct clinical courses and this poses a challenge to the systemic study of these diseases. The exact role of allogenic transplant in T- cell NHL is unknown. Methods: We looked at 45 pts who underwent an allogeneic HSCT for T cell lymphomas between Jan 2000 to Dec 2005. There were 18 males, 27 females. Median age at transplant was 32 (7–74). Histology was Mycosis fungoides /Sezary syndrome (SS/MF) n=10, T cell lymphobalstic leukemia (PTLL) n=16, PTCL including AILD and ALCL n=14, NKT cell n=5. Syngeneic donor 1, Sibs 31, MUD 13. Median time from diagnosis to transplant was 12.5 (3.6–88.3) mo. Source of stem cells BM 9, PBSCT 35, cord blood 1. Conditioning, fully ablative 29, reduced intensity 16. Median number of prior regimens MF/SS 5, PTLL 2, PTCL 3, NKTL 2 with only 2 pts with prior auto transplants. 18 pts were in remission at the time of transplant and 12 pts had induction failure. Results: Median follow up from transplant was 45.3 mo(0.7–64.7) with a 55.6%OS. Incidence of GVHD was Acute n= 27 extensive chronic n=20. Cause of death was transplant related in 16 pts with only 3 pts dying of disease progression. The overall survival is 61.2 % at 1 year,55.4% at 2 years and 48.5% at 5 years with a 5 year progression free survival at 48.5%. Based on different histologies, the results are 50.0% overall survival at 1 year and 40.0% at 2 and 5 years for SS/MF, 55.6% at 1 and 2 years for PTLL, 70.1% at 1 and 2 years and 52.6% at 5 years for PTCL and 80% at 1 year for NKT shown in fig 1 and 2. Conclusion: Allogenic transplant can result in long term survival for some patients with T cell NHL suggesting a graft vs lymphoma effect. Timing of transplant needs to be better defined. Most patients are heavily pretreated which may have resulted in more transplant related mortality with 11/20 pts dying of infection. Most patients did not have a prior auto transplant indicating early progression of disease after standard therapies defined for B cell malignancies. Overall Survival by Histology
 T-Cell Lymphoma with Allogenic Transplant
 Sample Size: 45 patients Overall Survival by Histology
 T-Cell Lymphoma with Allogenic Transplant
 Sample Size: 45 patients Progression-Free Survival by Histology
 T-Cell Lymphoma with Allogenic Transplant
 Sample Size: 45 patients Progression-Free Survival by Histology
 T-Cell Lymphoma with Allogenic Transplant
 Sample Size: 45 patients

Absolute monocyte count is a predictor of overall survival and progression-free survival in nodal peripheral T cell lymphoma

2019 ◽  
Vol 98 (9) ◽  
pp. 2097-2102
Author(s):  
Luís Alberto de Pádua Covas Lage ◽  
Débora Toshie Hamasaki ◽  
Frederico Rafael Moreira ◽  
Vanderson Rocha ◽  
Maria Cláudia Nogueira Zerbini ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Monocyte Count ◽  
Peripheral T Cell Lymphoma ◽  
Free Survival

scholarly journals Pretreatment Whole Blood Epstein-Barr Viremia Is a Prognostic Factor in Peripheral T-Cell Lymphoma

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4143-4143
Author(s):  
Yu Ri Kim ◽  
Soo-Jeong Kim ◽  
June-Won Cheong ◽  
Hyewon Lee ◽  
Haerim Chung ◽  
...  
Keyword(s):  
Overall Survival ◽  
Multivariate Analysis ◽  
T Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Free Survival

Abstract Abstract Peripheral T-cell lymphoma (PTCL) is the highly aggressive lymphoid malignancies, treatment outcome is very poor. There are increasing evidence about the role of Epstein-Barr virus (EBV) in PTCL. Because of its rarity, there was few studies about the prognostic factors incorporating EBV in PTCL. The aim of this study was to evaluate the role of EBV as prognostic factors in PTCL. We retrospectively reviewed the 174 PTCL patients (peripheral T-cell lymphoma, not otherwise specified; n=123, anaplastic large cell lymphoma; n=19, angioimmunoblastic T-cell lymphoma; n=26, enteropathy related T-cell lymphoma, n=5, hepatosplenic gammadelta T-cell lymphoma; n=1). Median age of the patients was 63 (20~94) years with 107 (61.5%) male patients. One-year OS and PFS was 55.5%, 37.5%, respectively. Stage 3 or 4 patients were 150 (86.2%). Bone marrow involvement were detected 73 (42.0%) patients among 163 available patients. For IPI scores, 29 (16.7%) patients were classified as low risk, 42 (24.1%) as low-intermediate risk, 57 (32.8%) as high-intermediate risk, and 46 (26.4%) as high risk. For PIT scores, 18 (11.1%) patients were classified in group 1, 41 (25.2%) in group 2, 58 (35.6%) in group 3, and 46 (28.2%) in group 4. Upfront autologous hematopoietic stem cell transplantation (n=17) improved OS and PFS (P=0.001 and P<0.001, respectively). In univariate analysis, poor performance status (ECOG ¡Ã2) (P <0.001 and P <0.001, respectively), low absolute lymphocyte counts (<1000/mm3) (P=0.022 and P=0.038, respectively), high ferritin (¡Ã1,000/mm3) (P =0.002 and P =0.002, respectively), EBV viremia in the whole blood (positive) (P=0.016 and P <0.001, respectively), low protein level (<6.3 g/dL) (P <0.001 and P <0.001, respectively) and low albumin level (<3.5 g/dL) (P =0.001 and P =0.001, respectively) were related with inferior OS and PFS. High international prognostic index (IPI) and prognostic index for PTCLu (PIT) were related with inferior OS and PFS (P<0.001, P=0.029 and P=0.019, P=0.278, respectively) (Figure 1A, 1B, 2A, 2B). In multivariate analysis, poor performance status, extranodal involvement more than one site and EBV viremia were related with OS and PFS in multivariate analysis. (P <0.001, P =0.024, P =0.001 and P =0.001, P=0.002, P=0.031, respectively). We made a new prognostic score model using statistically significant 3 variables in multivariate analysis: low, no adverse factors; intermediate, one factor; high, two or three factors. This model could identify three groups of patients for OS and PFS (Figure 3A,3B.) This study suggests that prognostic models including EBV for PTCL showed good risk classification. There will be need to investigate the mechanism of EBV and specific treatment strategy for EBV-related patients. These patients will be need to consider more effective therapeutic strategy to improve the poor survival in PTCL. Figure 1. Overall survival and progression free survival according to international prognostic index Figure 1. Overall survival and progression free survival according to international prognostic index Figure 2. Overall survival and progression free survival according to prognostic index for PTCLu Figure 2. Overall survival and progression free survival according to prognostic index for PTCLu Figure 3. Overall survival and progression free survival according to new prognostic model Figure 3. Overall survival and progression free survival according to new prognostic model Disclosures No relevant conflicts of interest to declare.

scholarly journals Progression-free survival at 24 months and subsequent survival of patients with extranodal NK/T-cell lymphoma: a China Lymphoma Collaborative Group (CLCG) study

Leukemia ◽  
2020 ◽  
Author(s):  
Yong Yang ◽  
Ying Wang ◽  
Xin Liu ◽  
Xia He ◽  
Li-Ling Zhang ◽  
...  
Keyword(s):  
T Cell ◽  
Large Scale ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Collaborative Group ◽  
Standardized Mortality Ratio ◽  
Free Survival ◽  
Excellent Outcome ◽  
Nk T Cell

Abstract Limited evidence supports the use of early endpoints to evaluate the success of initial treatment of extranodal NK/T-cell lymphoma (ENKTCL) in the modern era. We aim to analyze progression-free survival at 24 months (PFS24) and subsequent overall survival (OS) in a large-scale multicenter cohort of patients. 1790 patients were included from the China Lymphoma Collaborative Group (CLCG) database. Subsequent OS was defined from the time of PFS24 or progression within 24 months to death. OS was compared with age- and sex-matched general Chinese population using expected survival and standardized mortality ratio (SMR). Patients who did not achieve PFS24 had a median OS of 5.3 months after progression, with 5-year OS rate of 19.2% and the SMR of 71.4 (95% CI, 62.9–81.1). In contrast, 74% patients achieved PFS24, and the SMR after achieving PFS24 was 1.77 (95% CI, 1.34–2.34). The observed OS rate after PFS24 versus expected OS rate at 5 years was 92.2% versus 94.3%. Similarly, superior outcomes following PFS24 were observed in early-stage patients (5-year OS rate, 92.9%). Patients achieving PFS24 had excellent outcome, whereas patients exhibiting earlier progression had a poor survival. These marked differences suggest that PFS24 may be used for study design and risk stratification in ENKTCL.

scholarly journals OP0294 SJÖGREN’S SYNDROME ASSOCIATED LYMPHOMAS: CLINICAL DESCRIPTION AND 10-YEAR SURVIVAL

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 180-181
Author(s):  
L. Chatzis ◽  
V. Pezoulas ◽  
A. Goules ◽  
I. Stergiou ◽  
C. Mavragani ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Histologic Type ◽  
Single Center ◽  
Event Free Survival ◽  
Survival Curves ◽  
Free Survival ◽  
Kaplan Meier

Background:Sjögren’s Syndrome (SS) is a chronic systemic autoimmune disease of unknown etiology, carrying the highest lymphoma risk among autoimmune diseases, with significant impact on mortality and morbidity of patients.Objectives:To describe: i) the clinical phenotype of SS, ii) the histologic type, stage, treatment options regarding lymphomas and iii) the prognosis of patients with SS related lymphoproliferative disorders.Methods:Eight hundred and fifteen consecutive SS patients’ records from a single center fulfilling the 2016 ACR/EULAR were reviewed retrospectively for the purpose of this study. One hundred twenty-one patients with a diagnosis of non-Hodgkin Lymphoma (NHL) were identified and enrolled in the study population. Cumulative clinical, laboratory and histologic data were recorded and overall survival as well as event free survival curves were constructed using the Kaplan-Meier method. An event was defined as a disease progression, lymphoma relapse, treatment failure, histologic transformation, development of a 2nd lymphoma or death from any cause.Results:From 121 pSS patients with lymphoma the most common histologic type encountered was MALT lymphoma (92/121, 76,0%) followed by DLBCL (11/121, 9.0%) and NMZL (8/119, 6.6%). The remaining 10 patients had various lymphomas of B (follicular, lymphoplasmacytic, chronic lymphocytic leukemia} and T cell origin (peripheral T cell lymphoma not otherwise specified, primary cutaneous T cell lymphoma, angioimmunoblastic t-cell lymphoma). Permanent salivary gland enlargement (66.1%, 80/121), palpable purpura (34,7% 42/121), peripheral nervous involvement (9,9%, 12/121), interstitial lung disease (8,2%, 10/121) presence of serum cryoglobulins (38,7%, 43/111) and C4 hypocomplementemia (69,8% 81/116) present at least 1 year before the development of lymphoma were the main pSS related features. The median age at lymphoma diagnosis was 58 years old (range 29-82) while MALT lymphomas developed earlier compared to DLBCL from pSS diagnosis (8 vs 3 OR= 3.84, 95%CI: 0.29 to 10.46; p=0.0266). The commonest biopsy proven extranodal sites included the labial minor salivary (43,8% patients) and parotid glands (30,5%) while 11% of patients had more than 1 extranodal sites affected. Bone marrow involvement was evident in 24,3% of patients (29/119) while nodal involvement in 35,5% (42/118). The majority of patients (65%) had limited disease (stage I or II). A watch and wait therapeutic policy was chosen in 40 patients while the rest received rituximab with or without chemotherapy. The 10-year survival and event free rates were 79% and 45,5% for MALT lymphomas, 40,9% and 24,2% for DLBCL and 46% and 31% for NMZL respectively (Figure 1). The Mantel-Cox log-rank comparison of the overall survival curves revealed a statistically significant difference (p=0.0016) among lymphoma subtypes.Figure 1.Overall and event free survival of SS-associated lymphoma patients. A. Kaplan-Meier overall survival analysis. B. A Kaplan-Meier event free survival analysis.Conclusion:This is the largest single center series of SS- associated lymphoma patients, providing a detailed description of SS and lymphoma related features, combined with a 10-year survival and event free curves for the first time in the literature.Disclosure of Interests:None declared.

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