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

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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Event-Free Survival at 12 Months and Subsequent Overall Survival in Patients with Peripheral T-Cell Lymphoma

Blood ◽

10.1182/blood.v126.23.1501.1501 ◽

2015 ◽

Vol 126 (23) ◽

pp. 1501-1501

Author(s):

Matthew J Maurer ◽

Fredrik Ellin ◽

James Cerhan ◽

Stephen Ansell ◽

Brian K Link ◽

...

Keyword(s):

Overall Survival ◽

T Cell ◽

Research Funding ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Newly Diagnosed ◽

Event Free Survival ◽

Free Survival ◽

T Cell Lymphomas

Abstract Background: Peripheral T-Cell lymphomas (PTCLs) constitute approximately 10% of lymphoid malignancies and consist of several distinct entities based on pathologic and clinical characteristics. With the exception of a few subtypes (e.g., ALK-positive anaplastic large cell lymphoma (ALCL) and some primary cutaneous or leukemic forms of PTCL), a majority of PTCLs are aggressive as characterized by poor treatment response, rapid disease progression and poor overall survival. We have shown that landmark timepoints of event-free survival after diagnosis can stratify subsequent overall survival (OS) in diffuse large B-cell and follicular lymphoma. Here we evaluate this approach in newly diagnosed aggressive PTCLs treated with anthracyline-based or related chemotherapy. Methods. Newly diagnosed PTCL patients were prospectively enrolled in the University of Iowa/Mayo Clinic Lymphoma SPORE Molecular Epidemiology Resource (MER) from 2002-2012. Clinical data were abstracted from medical records using a standard protocol. For this analysis, we included patients receiving anthracycline-based or other multiagent chemotherapy for the following PTCL subtypes: ALK-negative ALCL (N=24); angioimmunoblastic T-cell lymphoma (AITL, N=34); PTCL, not otherwise specified (NOS; N=60); enteropathy-associated T-cell lymphoma (EATL, N=8); extranodal NK/T-cell lymphoma, nasal type (ENKTL, N=11); and hepatosplenic T-cell lymphoma (HSTCL, N=1). Patients were prospectively followed, and event-free survival (EFS) was defined as time from diagnosis to progression, re-treatment, or death due to any cause. Landmark EFS timepoints were assessed at 12 (EFS12) and 24 (EFS24) months after the date of diagnosis. Subsequent OS was defined as time from a specific endpoint (diagnosis, event or EFS landmark). Replication was performed in a population-based cohort of T-cell lymphomas diagnosed from 2000-2009 from the Swedish Lymphoma Registry. Results. 138 eligible patients were enrolled in the MER from 2002-2012, the median age at diagnosis was 58 years (range, 19-88), 66% were male, 73% had Stage III-IV disease, and 33% had IPI 0-1. At a median follow-up of 47 months (range 11-120), 87 patients (63%) had an event and 70 patients (51%) had died. From diagnosis, only 60 patients were event-free at 12 months (EFS12 45%). Patients who failed to achieve EFS12 had a poor subsequent OS from event (median OS = 6.8 months, 95% CI: 5.3-14.0, figure 1). In contrast, patients who achieved EFS12 had a favorable subsequent OS (median unreached, figure 2). Of the 427 eligible patients in the Swedish registry, the median age at diagnosis was 66 years (range, 18-88), 63% were male, 68% had Stage III-IV disease, and 25% had IPI 0-1. PTCL subtypes were: ALK-negative ALCL (N=89); AITL (N=80); PTCL, NOS (N=183); EATL (N=44); ENKTL (N=24); and HSTCL (N=7). At a median follow-up of 86 months (range 40-158), 333 patients (79%) had an event and 316 patients (74%) had died. From diagnosis, 183 patients were event-free at 12 months (EFS12 44%). Similar to the MER cohort, Swedish patients failing EFS12 had poor subsequent survival (median OS = 3.7 months, 95% CI: 2.9-5.3, figure 1). Swedish patients achieving EFS12 had a favorable subsequent OS (median OS = 89 months, figure 2). Similar results were obtained when conducting landmark analysis at 24 months after diagnosis (EFS24). Conclusion. Relapse and re-treatment events within the first 12 months of diagnosis are associated with very poor OS in PTCL treated with anthracyclines or related chemotherapy, while patients achieving EFS12 have encouraging subsequent OS. Stratifying patients into prognostically distinct subsets using EFS12 may help focus biologic and biomarker studies. EFS12 has potential as an early endpoint for studies of newly diagnosed PTCL. Further investigation of determinants related to post-EFS12 survival is needed. Disclosures Maurer: Kite Pharma: Research Funding. Cerhan:Kite Pharma: Research Funding. Ansell:Bristol-Myers Squibb: Research Funding; Celldex: Research Funding. Link:Genentech: Consultancy, Research Funding; Kite Pharma: Research Funding. Thompson:Kite Pharma: Research Funding. Relander:Respiratorius: Patents & Royalties: valproate for DLBCL.

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

Blood ◽

10.1182/blood.v124.21.1706.1706 ◽

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.

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

Blood ◽

10.1182/blood-2019-125666 ◽

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.

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

Blood ◽

10.1182/blood.v108.11.3026.3026 ◽

2006 ◽

Vol 108 (11) ◽

pp. 3026-3026 ◽

Cited By ~ 1

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

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Outcome of Patients with Peripheral T-Cell Lymphoma Undergoing Allogeneic Stem Cell in British Columbia

Blood ◽

10.1182/blood.v128.22.5852.5852 ◽

2016 ◽

Vol 128 (22) ◽

pp. 5852-5852

Author(s):

Musa Alzahrani ◽

Kerry J Savage ◽

Cynthia L. Toze ◽

Laurie H Sehn ◽

Raewyn Broady ◽

...

Keyword(s):

British Columbia ◽

T Cell ◽

Research Funding ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Primary Therapy ◽

Peripheral T Cell Lymphoma ◽

Free Survival ◽

Kaplan Meier

Abstract Background: Peripheral T-cell lymphomas (PTCL) are a rare and heterogeneous group of non-Hodgkin lymphomas (NHLs) that accounts for approximately 10% of all aggressive NHLs in Western countries. The optimal management remains unclear, however, given the poor outcome, allogeneic transplant (allo-SCT) has been integrated into the front-line treatment for some rare extranodal subtypes as well in relapsed/refractory setting. We report our provincial experience of the outcome of patients with PTCL who have undergone allo-SCT at the British Columbia Cancer Agency (BCCA). Methods: The Leukemia/BMT Program of British Columbia database and the BCCA Lymphoid Cancer Database were searched to identify all patients diagnosed with PTCL who have undergone allo-SCT between November 1990 and January 2016. Overall survival and relapse free survival were estimated using the Kaplan-Meier method. Results: We identified 36 cases of PTCL patients who have undergone allogeneic transplant from a median time of 11 months from primary diagnosis (range 4-64) with the following clinical features: median age at transplant was 45 years (range 16-58 years); 24 (67%) were male; 32 (89%) patients had advanced stage disease; 22 (61%) had B-symptoms at diagnosis. Bone marrow involvement detected in 13/34 (38%) patients. Histological diagnosis based on the WHO 2008 classification were: Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) n=15 (42%); anaplastic large cell lymphoma (ALCL) n=7 (19%) out of which three were ALK positive, angioimmunoblastic T-cell lymphoma (AITL) n=6 (17%), hepatosplenic T-cell lymphoma n=5 (14%), enteropathy associated T-cell lymphoma type I n=1, advanced primary cutaneous gamma-delta T-cell lymphoma n=1 and Sezary syndrome n=1. Ten patients (28%) underwent allo-SCT as part of planned primary therapy after achieving their first remission [6 were in complete remission 1 (CR1) and 4 were in partial remission 1 (PR1)], whereas 26 patients (72%) underwent allo-SCT for relapsed/refractory disease. CHOP was administered in 19 patients (53%) as the primary therapy. 17 (47%) patients received alternative chemotherapy regimen due to patient and/or physician preference. The clinical status at the time of transplantation was CR in 12 patients (33%), relapsed sensitive disease n=10 (28%), relapsed untreated n=5 (14%), partial remission (PR) in 5 (14%), primary progressive disease n=3 (8%) and relapsed resistant disease n=1 (3%). Thirty two patients (89%) underwent myeloablative conditioning, 4 (11%) underwent reduced intensity conditioning (RIC). The conditioning regimens included: cyclophosphamide/TBI n=24 (67%), busulfan/cyclophosphamide n=5 (14%), fludarabine containing reduced intensity n=4 (11%), and other regimens n= 3 (8%). With a median follow-up of alive patients from the time of allo-SCT of 69 months (range 1-186 months). At last follow-up, 17 (47%) patients have died, 6 from disease relapse, 5 from graft vs host disease (GVHD), 2 from regimen related toxicity and 4 from other causes. Nineteen patients (53%) still alive at last follow up post-transplant of which 14 (39%) still in remission. The 5-year event free survival (EFS) and overall survival (OS) from the time of allo-SCT of all patients were 43% and 63%, respectively. For PTCL-NOS the 5-year EFS and OS were 52% and 69%, respectively. Table 1 summarizes the patients' characteristics. Figures 1 and 2 shows the Kaplan-Meier curves for OS and EFS respectively. Conclusion: Allo-SCT can be effective strategy in select patients with relapsed/refractory PTCL and those with high risk histologies in the upfront setting PTCL with an acceptable toxicity. Disclosures Toze: Roche Canada: Research Funding. Sehn:roche/genentech: Consultancy, Honoraria; amgen: Consultancy, Honoraria; seattle genetics: Consultancy, Honoraria; abbvie: Consultancy, Honoraria; TG therapeutics: Consultancy, Honoraria; celgene: Consultancy, Honoraria; lundbeck: Consultancy, Honoraria; janssen: Consultancy, Honoraria. Scott:NanoString Technologies: Patents & Royalties: named inventor on a patent for molecular subtyping of DLBCL that has been licensed to NanoString Technologies. Villa:Lundbeck: Honoraria; Roche: Honoraria, Research Funding; Celgene: Honoraria. Connors:NanoString Technologies: Research Funding; F Hoffmann-La Roche: Research Funding; Bristol Myers Squib: Research Funding; Seattle Genetics: Research Funding; Millennium Takeda: Research Funding. Song:Janssen: Honoraria; Otsuka: Honoraria; Celgene: Honoraria, Research Funding.

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Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project

Blood ◽

10.1182/blood-2010-09-310342 ◽

2011 ◽

Vol 117 (12) ◽

pp. 3402-3408 ◽

Cited By ~ 228

Author(s):

Dennis D. Weisenburger ◽

Kerry J. Savage ◽

Nancy Lee Harris ◽

Randy D. Gascoyne ◽

Elaine S. Jaffe ◽

...

Keyword(s):

Overall Survival ◽

T Cell ◽

Cell Lymphoma ◽

International Prognostic Index ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Bulky Disease ◽

Free Survival ◽

Not Otherwise Specified ◽

Pathologic Features

Abstract The International Peripheral T-cell Lymphoma Project is a collaborative effort to better understand peripheral T-cell lymphoma (PTCL). A total of 22 institutions submitted clinical and pathologic material on 1314 cases. One objective was to analyze the clinical and pathologic features of 340 cases of PTCL, not otherwise specified. The median age of the patients was 60 years, and the majority (69%) presented with advanced stage disease. Most patients (87%) presented with nodal disease, but extranodal disease was present in 62%. The 5-year overall survival was 32%, and the 5-year failure-free survival was only 20%. The majority of patients (80%) were treated with combination chemotherapy that included an anthracycline, but there was no survival advantage. The International Prognostic Index (IPI) was predictive of both overall survival and failure-free survival (P < .001). Multivariate analysis of clinical and pathologic prognostic factors, respectively, when controlling for the IPI, identified bulky disease (≥ 10 cm), thrombocytopenia (< 150 × 109/L), and a high number of transformed tumor cells (> 70%) as adverse predictors of survival, but only the latter was significant in final analysis. Thus, the IPI and a single pathologic feature could be used to stratify patients with PTCL-not otherwise specified for novel and risk-adapted therapies.

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