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

Blood ◽  
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.

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.

Clinicopathological Characteristics and Clinical Course of CD8 Expressing Primary Cutaneous Peripheral T-Cell Lymphomas (CTCL) - Retrospective Case Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5213-5213
Author(s):  
Deniz Peker ◽  
Yizhou Zhang ◽  
Young Yu ◽  
Zhigang Zhao ◽  
Yafei Wang ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Clinical Outcome ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Clinicopathological Characteristics ◽  
Therapeutic Strategies ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas

Abstract Abstract 5213 Background: CD8-positive primary cutaneous T cell lymphomas (CTCL) are rare disorders and mainly include primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (AECTL) and CD8+ variant mycosis fungoides (MF). In contrast to primary cutaneous CD8+ AECTL, which frequently exhibits strikingly aggressive and unfavorable clinical behavior, CD8+ MF shows debatable clinical course, from an indolent to aggressive behavior. As previously reported, the indolent subtype CD8+ MF occur more frequently in pediatric group, while both clinical subtypes have been observed in adults. Albeit single case studies or small case series have been reported in the literature, it still lacks a large scale of study to enlighten the clinicopathological aspects of CD8+ primary CTCLs, in order to develop the appropriate therapeutic strategies. This study aims to retrospectively review these two entities to demonstrate their clinicopathologic characteristics and to correlate them with the clinical outcome. Design: The hematopathology files from H. Lee Moffitt Cancer Center & Research Institute (PATHNET) and Tianjian Cancer Research Institute were retrieved. The patients with a primary diagnosis of CD8 expressing primary CTCLs, diagnosed and treated between January 2004 and June 2011, were included. Cutaneous involvement by systemic peripheral T-cell lymphoma, primary cutanous gamma delta T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma were excluded. The corresponding patient demographics, laboratory datas, therapeutic strategies and the clinical outcomes were reviewed. All available histology slides, along with all of the ancillary study results were reviewed and correlated with the clinical outcome. Results: Total of 10 cases were included based on the confirmed histomorphological diagnosis. Cases were divided into two groups: 1) CD8+ MF (n=5) and 2) CD8+ non-MF (n=5) including 2 cases with definitive diagnosis of AECTL and 3 cases diagnosed as CD8-positive primary cutaneous T cell lymphoma, not further classifiable. Clinicopathological characteristics including patients' demographic data, diagnosis, site of involvement, treatment, duration of follow up and clinical outcomes are summarized in table 1. The overall survival time for CD8+CTCLs, non-MF type (excluding 1 patient with lost follow up) varied from 5 to 90 months (averaging 20.5 months) while it was shorter in CD8+ MF, 12.6 months (5 to 23 months). Of note, 1 patient with AECTL expired shortly after diagnosis, within 3 months, however; the other one received allogeneic hematopoietic stem cell transplant (allo-HSCT) and has been alive up to date. Conclusion: CD8-positive CTCLs remain a diagnostic challenge. CD8+ MF in adults exhibit dual growth patterns: localized or systemically disseminated disease. The latter could have a very short median overall survival regardless of the aggressive therapies. Allo-HSCT might be beneficial to those with AECTL. Larger series of CD8+ MF should be investigated for molecular gene profiling in order to establish genetic, molecular and phenotypic parameters not only to separate the indolent form from the aggressive subtype, but also to distinguish it from primary cutaneous CD8-positive AECTL. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Long Term Outcomes of Patients with Aggressive T-Cell Non-Hodgkin Lymphoma Undergoing Allogeneic Stem Cell Transplantation: Retrospective Results from a Single Center

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4623-4623
Author(s):  
Amandeep Salhotra ◽  
Liana Nikolaenko ◽  
Lu Chen ◽  
NI-Chun Tsai ◽  
Diane Lynne Smith ◽  
...  
Keyword(s):  
Overall Survival ◽  
Stem Cell ◽  
T Cell ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Chronic Gvhd ◽  
T Cell Lymphoma ◽  
Long Term Follow Up

Background: Mature T cell and NK cell neoplasms collectively known as peripheral T-cell lymphomas (PTCL) comprise 15-20% of Non-Hodgkin lymphomas in adults and have a poor prognosis with a 5-year survival of less than 30% for the most aggressive subtypes. Allogeneic HCT (allo-HCT) is offered to eligible patients as a potentially curative modality in the salvage setting or in high risk patients to consolidate an initial response to frontline therapy. There are few studies that report clinical outcomes derived from large sample size and long-term follow up data. Methods: We retrospectively reviewed medical records of 87 consecutive patients with PTCL including transformed mycosis fungoides and NK/T-cell lymphoma without prior autologous transplant who underwent allo-HCT at City of Hope from January 2000 to June 2018 after IRB approval was obtained. Descriptive statistics were used to summarize baseline patient demographic, treatment, and disease characteristics. Kaplan-Meier curves and the log-rank test were used to evaluate the overall survival (OS) and progression-free survival (PFS). Cumulative incidences of time to relapse and time to non-relapse mortality (NRM) were calculated with relapse and NRM as competing risks. Cumulative incidences of acute and chronic GVHD were calculated as time to onset of GVHD with relapse and death as competing events for GVHD. Results: 87 patients were included for the analysis. Median age at the time of allo-HCT was 49 years (range 2-70 years). Histologies were PTCL-NOS (n=21); transformed CTCL (n=19); NK T-cell lymphoma (n=17); AITL (n=15), ALCL (n=7); gamma delta T-cell lymphoma (n=6) and other rare subtypes (n=2). None of the patients had a prior auto transplant. 42 patients (48%) received myeloablative conditioning, with the majority of patients receiving FTBI based conditioning (n=39) and three patients received BEAM regimen for conditioning. 45 patients (52%) received reduced intensity conditioning; fludarabine/melphalan based-conditioning was the most common regimen used (n=39). Sibling HCT was performed in 47 patients (54%), while MUD HCT was performed in 36 patients (41%) with fully matched HLA unrelated donor in 15 (17%) and HLA mismatched in 21 (24%) patients; 4 (5%) received haploidentical HCT. GVHD prophylaxis consisted of tacrolimus/sirolimus (n=54), tacrolimus/sirolimus/MTX (n=11), tacrolimus or cyclosporine/MTX (n=7), tacrolimus or cyclosporine/MMF (n=7), post-transplant cyclophosphamide/tacrolimus/MMF (n=5) and other (n=3). Source of stem cells was PBSC in 77 (88%), bone marrow in 5 (6%), and cord blood in 5 (6%) patients. At the time of allo-HCT, there were a total of 25 (29%) patients in complete remission (CR1 n=15, CR2+ n=10), 25 (29%) patients in partial remission 22 (25%) with induction failure and 14 (16%) with relapsed disease. The median follow-up among survivors was 6.9 years (range 1.1-15.5). The 5- and 10-year PFS was 47% (95% CI: 36%-58%) and 38% (95% CI: 26%-50%), respectively. The 5- and 10-year OS was 53% (95% CI: 41%-63%) and 42% (95% CI: 29%-54%), respectively (Fig.1). Relapses at 5 and 10 years were both 24% (95% CI: 16%-34%), while NRM at 5 and 10 years was 28% (95% CI: 19%-39%) and 37% (95% CI: 25%-50%), respectively. At day 100 after allo-HCT, the rates of acute GVHD grade II-IV were 41% (95% CI: 30%-51%) and grade III-IV of 16% (95%CI: 9%-25%). Chronic GVHD rates at 3 years were 62% (95% CI: 51%-72%), with extensive GVHD of 55% (95% CI: 44%-65%). On univariate analysis, age (> 60 or not), sex, TBI-based conditioning, donor type (MSD vs MUD), stem cell source or remission status prior to allo-HCT did not predict for overall survival in our study. Conclusions: Our results constitute the largest reported single-institution series with a long-term follow-up on allo-HCT outcomes in patients with aggressive T-cell NHL. The 5-year PFS and OS of 47 and 53%, respectively, are encouraging for the high-risk T-cell NHL patients with limited treatment options. Disclosures Salhotra: Celgene: Other: Research Support; Kadmon Corporation: Other: Non paid consultant. Popplewell:City of Hope: Employment. Herrera:Adaptive Biotechnologies: Consultancy; Bristol-Myers Squibb: Consultancy, Research Funding; Gilead Sciences: Consultancy, Research Funding; Seattle Genetics: Consultancy, Research Funding; AstraZeneca: Research Funding; Merck: Consultancy, Research Funding; Genentech, Inc.: Consultancy, Research Funding; Pharmacyclics: Research Funding; Immune Design: Research Funding; Kite Pharma: Consultancy, Research Funding. Mei:Seattle Genetics, Inc.: Research Funding. Zain:spectrum: Honoraria; Seattle Genetics: Honoraria, Speakers Bureau.

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

Blood ◽  
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.

scholarly journals CD70 Expression in Mature T-Cell Lymphomas

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4493-4493
Author(s):  
Mario L. Marques-Piubelli ◽  
Luisa Solis Soto ◽  
Swaminathan Padmanabhan Iyer ◽  
Jason Sagert ◽  
Minh Thu Pham ◽  
...  
Keyword(s):  
T Cell ◽  
Mycosis Fungoides ◽  
Research Funding ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Advanced Age ◽  
Oncology Research ◽  
T Cell Lymphomas ◽  
Pathologic Classification

Abstract Introduction Mature T-cell lymphomas (TCLs) represent about 15% of the non-Hodgkin lymphomas in U.S. and are a heterogeneous group of neoplasms. TCLs usually have poor outcomes and are resistant to conventional chemotherapy regimens. Therefore, there is a need for identifying biomarkers that could translate into effective novel treatments. CD70 is the TNF superfamily ligand of CD27 and it is being explored as a potential target in CAR-T therapy against TCLs and other neoplasms. Although CD70 expression has been documented in TCL cell lines, thus providing a potential rationale for its use as a therapeutic target, the expression of CD70 among the most frequent TCL subtypes in patient samples has not been previously evaluated. Methods Patients with de novo and/or relapsed mature TCLs diagnosed between 01/2010 and 06/2020 and with available tissue sections were included in the study. The assessment of CD70 expression was performed by immunohistochemistry (IHC) using a novel proprietary antibody developed by CRISPR Therapeutics. CD70 expression was scored using % of expression, intensity (negative, +1, +2, and +3) and H-score {H-score = [(%positive cells intensity 1+) x 1] + [(%positive cells intensity 2+) x 2] + [(%positive cells intensity 3+) x 3]} in neoplastic cells (Figure 1). Clinicopathologic characteristics were collected retrospectively and included age, sex, staging, biopsy site, WHO pathologic classification, immunophenotype, presence of other malignancies, treatment status, disease progression/relapsed, and follow-up. These characteristics were compared with CD70 expression using ANOVA or non-parametric analysis. Overall-survival (OS) was estimated using Kaplan-Meier method and compared using log-rank. A p< 0.05 was considered statistically significant. Results One hundred thirty-six patient samples representing the major subtypes of the 4 categories of mature TCLs were included [nodal TCLs (n=64; including PTCL Th1, and Th2 subtypes and angioimmunoblastic T cell lymphoma - AITL), extranodal (n=35; including primary intestinal lymphomas), cutaneous (n=24; including mycosis fungoides (MF) with large cell transformation), and leukemic (n=13; including adult T cell leukemia/lymphoma)]. Most patients were male (58.8%), in the 6 th or 7 th decade (47.1%), with advanced stage IV (55.7%), no previous malignancies (69.9%) and previously treated (57.2%); 59 of which (88%) received two or more lines of treatment. The median expression of CD70 was 40% (ranged from 0 to 100%) and the median H-score was 110 (ranged from 0 to 300) and was significantly higher (p= 0.006) in peripheral T-cell lymphoma, NOS, primary cutaneous TCLs (non-mycosis fungoides), and AITL (180, 150, and 150, respectively) (Figure 1). The expression of CD70 was more frequent in nodal and extranodal subtypes (including skin), compared to leukemic TCLs (p= 0.005). The expression of CD70 was associated with advanced age at the diagnosis (p= 0.003), CD2 expression (p= 0.01), CD3 expression (p= 0.001), CD16 negativity (p= 0.03), and absence of ALK-1 expression (p= 0.005). After a median follow-up of 19 months (range: 1 - 300 months), 42% of the patients died of disease. The median OS was 76 months (CI95, 38.5 - 113.4 months) and it was not associated with CD70 expression. Conclusions CD70 was highly expressed in most mature TCLs and it was negative in most ALK-positive ALCL. Its expression was associated with nodal and extranodal subtypes, advanced age, expression of CD2, CD3, and negativity for CD16. Therefore, CD70 can be used as a potential biomarker and a target in clinical trials of patients with mature TCLs. Figure 1 Figure 1. Disclosures Iyer: CRISPRX: Research Funding; Seattle Genetics: Research Funding; Rhizen: Research Funding; Merck: Research Funding; Legend: Research Funding; Innate: Research Funding; Spectrum: Research Funding; Trillium: Research Funding; Astra Zeneca: Research Funding; Yingli: Research Funding; Cyclacel: Research Funding. Sagert: CRISPR Therapeutics: Current Employment. Pham: CRISPR Therapeutics: Current Employment. Tipton: CRISPR Therapeutics: Current Employment. Vega: i3Health, Elsevier, America Registry of Pathology, Congressionally Directed Medical Research Program, and the Society of Hematology Oncology: Research Funding; CRISPR Therapeutics and Geron: Research Funding.

scholarly journals An International Assessment of Event-Free Survival at 24 Months (EFS24) and Subsequent Survival in Peripheral T-Cell Lymphoma

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 920-920
Author(s):  
Matthew J Maurer ◽  
Fredrik Ellin ◽  
Line Srour ◽  
Mats Jerkeman ◽  
Nabila Nora Bennani ◽  
...  
Keyword(s):  
T Cell ◽  
Research Funding ◽  
Stem Cell Transplant ◽  
Cell Lymphoma ◽  
Autologous Stem Cell Transplant ◽  
T Cell Lymphoma ◽  
Cell Transplant ◽  
Event Free Survival ◽  
Free Survival ◽  
First Remission

Abstract Background: Peripheral T-cell lymphomas (PTCLs) comprise a group of non-Hodgkin lymphomas (NHLs) of mature T-cell origin with generally aggressive clinical behavior. Most systemic PTCLs are treated with anthracycline-based combination chemotherapy; however, outcomes remain relatively poor for most subtypes except ALK-positive anaplastic large cell lymphoma (ALCL). We have used landmark analyses based on event-free survival (EFS) to identify clinically useful endpoints in B-cell NHLs, with EFS at 24 (EFS24) months identified to stratify overall survival (OS) in aggressive B-cell NHL. Here we examined the ability of EFS24 to predict subsequent OS in a large, multinational PTCL cohort. Methods: A cohort of newly diagnosed PTCL patients treated with curative intent combination chemotherapy regimens was assembled from the University of Iowa/Mayo Clinic SPORE Molecular Epidemiology Resource (MER), Swedish Lymphoma Registry (SWE), and British Columbia Cancer Agency (BCCA). Subtypes included ALK-negative ALCL, angioimmunoblastic T-cell lymphoma (AITL), PTCL, not otherwise specified (NOS), enteropathy-associated T-cell lymphoma (EATL), extranodal NK/T-cell lymphoma, nasal type (ENKTL), and hepatosplenic T-cell lymphoma (HSTCL). Patients were followed based on local institution guidelines, and EFS was defined as time from the date of pathologic diagnosis to progression, re-treatment, or death due to any cause. EFS24 was defined as being alive and event-free 24 months from diagnosis. Subsequent OS was defined as time from achieving EFS24 (24 months from diagnosis) or time from progression in patients failing to achieve EFS24 (progression within 24 months of diagnosis). OS was compared to the age-, sex-, and country-matched general population using United States, Sweden, and British Columbia rate tables via standardized mortality ratios (SMR) and expected survival. Results: 775 patients diagnosed from 2000-2012 were included in the combined analysis with diagnosis by the WHO classification at the respective institutions (MER=138, SWE=422, BCCA=215). Median age at diagnosis was 64 years (range 18-89) and 63% were male. Patient characteristics are summarized in the table. 736 (95%) received anthracyline-based therapy at diagnosis. At a median follow-up of 77 months (range 1-185), 516 patients (67%) had died. The percentage of patients achieving EFS24 was similar across the 3 cohorts (MER=39%, SWE=35%, BCCA= 36%, combined=36%). Median survival after progression within the first 24 months was only 4.9 months (95% CI: 3.8-5.9), with a 5-year OS of 11% and SMR of 46.4 (95% CI: 41.8-51.3). In contrast, median survival after achieving EFS24 was not reached, with a 5-year OS of 78% (95% CI: 73%-84%). This was inferior to the expected survival of 92% in the age-, sex-, and country matched population (SMR=3.16; 95% CI: 2.48-3.98). In EFS24 subgroup analyses, more favorable outcomes were observed in younger patients (age <=60 years, N=137, 5-year survival of 91% vs. 98% expected) and in patients receiving autologous stem cell transplant in first remission (N=72, 5-year survival of 88% vs. 96% expected). Patients achieving EFS24 who were not transplanted in first remission (N=189) had a 5-year survival of 74% vs. 90% expected. Conclusions: Assessment of EFS24 stratifies subsequent outcome in PTCL. Patients with early relapse from PTCL have extremely poor outcomes. However, over one-third of patients with PTCL remain in remission two years from diagnosis after initial chemotherapy and have encouraging OS rates, although survival remains significantly worse than the matched general population. Subset analysis suggests that younger patients and patients who receive autologous stem-cell transplant in first CR have 5-year survival rates that approach 90%, though still below the expected survival of the background population. The marked differences in OS after failing or achieving EFS24 in PTCL patients suggest that this endpoint may be useful for patient counseling, as a clinical trial endpoint, and as an endpoint to assess novel biomarkers for risk stratification. Table Table. Figure 1. Figure 1. Figure 2. Figure 2. Disclosures Maurer: Celgene: Research Funding; Kite Pharma: Research Funding. Jerkeman:Mundipharma: Research Funding; Amgen: Research Funding; Janssen: Research Funding; Gilead: Research Funding; Celgene: Research Funding. Connors:Seattle Genetics: Research Funding; Millennium Takeda: Research Funding; NanoString Technologies: Research Funding; F Hoffmann-La Roche: Research Funding; Bristol Myers Squib: Research Funding. Ansell:BMS, Seattle Genetics, Merck, Celldex and Affimed: Research Funding.

scholarly journals Autologous Stem Cell Transplantation in First Complete Remission May Not Extend Progression Free Survival in Patients with ALK-Negative Peripheral T Cell Lymphoma

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3183-3183 ◽  
Author(s):  
Clinton Yam ◽  
Daniel J. Landsburg ◽  
Xinyi Lin ◽  
Anthony Mato ◽  
Jakub Svoboda ◽  
...  
Keyword(s):  
T Cell ◽  
Induction Chemotherapy ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Primary Therapy ◽  
Free Survival

Abstract Background: Peripheral T cell lymphoma (PTCL) is associated with a poor prognosis when treated with conventional front-line chemotherapy. Although consolidative autologous stem cell transplantation (ASCT) has been reported to improve progression free survival (PFS) when compared to historical controls, there is limited data comparing outcomes in PTCL patients undergoing ASCT versus active observation (AO) in first complete remission (CR1) following CHOP-based induction chemotherapy. Here we compare outcomes in this population at a single institution. Methods: We conducted a retrospective cohort study of PTCL patients treated from 1/1/2007-12/31/2014 and collected data through 7/1/2015. Patients with ALK+ anaplastic large cell lymphoma and cutaneous T cell lymphoma were censored from this analysis. Response to induction chemotherapy was verified through a review of clinical notes and, where available, relevant imaging studies. Therapy was given at the discretion of the treating physician. Our primary outcome measure was PFS, defined as the time from diagnosis to relapse or most recent follow up. Kaplan Meier survival analysis and the log rank test were used to compare outcomes between patients who underwent consolidative ASCT versus those who initiated AO. Results: We identified 105 PTCL patients who received a CHOP-based regimen as first line therapy. 52.4% (55/105) achieved CR1. Of these, 28 initiated AO, 20 underwent consolidative ASCT, 3 underwent consolidative radiation, 1 initiated weekly methotrexate and 3 were lost to follow up. There were no statistically significant differences in age, distribution of PTCL subtypes, IPI scores, stage, B symptoms, LDH, ECOG performance status and choice of primary therapy between the AO group and the ASCT group (Table 1). 28.6% (8/28) and 30.0% (6/20) of patients received etoposide as part of primary therapy in the AO and ASCT group respectively. After a median follow up duration of 22 months, median PFS for the AO and ASCT groups were 15.8 months (95% CI 7.8-23.7) and 12.8 months (95% CI 0-34.0) respectively (Figure 1, p=0.72). Conclusion: In this study, consolidative ASCT was not associated with significantly improved PFS as compared to observation alone for PTCL patients achieving CR1 following CHOP-based induction chemotherapy. Our institutional experience highlights the poor prognosis of patients with PTCL despite an excellent initial response to cytotoxic chemotherapy. With that in mind, novel approaches, including the use of non-cytotoxic agents as part of first-line or consolidative therapy, should be considered in the context of a clinical trial. Table 1. Baseline characteristics and choice of primary therapy AO (n=28) ASCT (n=20) P value Median age - years (range) 61.9 (32.8-78.3) 56.9 (21.4-71.9) 0.07 PTCL subtype PTCL NOS1 - no. (%) 16 (57.1) 6 (30.0) 0.41 AITL2 - no. (%) 5 (17.9) 6 (30.0) ALK- ALCL3 - no. (%) 5 (17.9) 3 (15.0) EATL4 - no. (%) 0 1 (5.0) ATLL5 - no. (%) 1 (3.6) 0 HSTCL6 - no. (%) 1 (3.6) 1 (5.0) Unknown - no. (%) 0 3 (15.0) IPI score at diagnosis 0-1 - no. (%) 5 (17.9) 3 (15.0) 1.00 2-3 - no.(%) 13 (46.4) 10 (50.0) 4-5 - no. (%) 3 (10.7) 2 (10.0) Unknown - no. (%) 7 (25.0) 5 (25.0) Stage at diagnosis I/II - no. (%) 7 (25.0) 4 (20.0) 0.73 III/IV - no. (%) 19 (67.9) 16 (80.0) Unknown - no. (%) 2 (7.1) 0 B symptoms at diagnosis Yes - no. (%) 17 (60.7) 13 (65.0) 1.00 No - no. (%) 9 (32.1) 7 (35.0) Unknown - no. (%) 2 (7.1) 0 LDH at diagnosis Elevated - no. (%) 12 (42.9) 11 (55.0) 0.46 Not elevated - no. (%) 7 (25.0) 3 (15.0) Unknown - no. (%) 9 (32.1) 6 (30.0) ECOG PS at diagnosis 0-1 - no. (%) 17 (60.7) 17 (85.0) 0.26 ≥2 - no. (%) 7 (25.0) 2 (10.0) Unknown - no. (%) 4 (14.3) 1 (5.0) Primary therapy CHOP - no. (%) 18 (64.3) 13 (65.0) 0.82 CHOP/Etoposide - no. (%) 7 (25.0) 6 (30.0) Other - no. (%) 3 (10.7)7 1 (5.0)8 1Peripheral T Cell Lymphoma, Not Otherwise Specified; 2Angioimmunoblastic T Cell Lymphoma; 3ALK negative Anaplastic Large Cell Lymphoma; 4Enteropathy Associated T Cell Lymphoma; 5Adult T Cell Leukemia/Lymphoma; 6Hepatosplenic T Cell Lymphoma; 72 patients received HyperCVAD and 1 patient received HyperCVED; 81 patient received CHOP alternating with DHAP Disclosures Mato: AbbVie: Consultancy, Research Funding; TG Therapeutics: Research Funding; Gilead: Consultancy, Research Funding; Pronai Pharmaceuticals: Research Funding; Genentech: Consultancy; Janssen: Consultancy; Pharmacyclics: Consultancy, Research Funding; Celgene Corporation: Consultancy, Research Funding. Svoboda:Celgene: Research Funding; Immunomedics: Research Funding; Celldex: Research Funding; Seattle Genetics: Research Funding. Loren:Merck: Research Funding. Frey:Novartis: Research Funding. Porter:Novartis: Other: IP interest, Research Funding; Genentech: Other: Spouse employment. Schuster:Genentech: Consultancy; Pharmacyclics: Consultancy, Research Funding; Celgene: Consultancy, Research Funding; Hoffman-LaRoche: Research Funding; Janssen: Research Funding; Gilead: Research Funding; Nordic Nanovector: Membership on an entity's Board of Directors or advisory committees; Novartis: Research Funding.

scholarly journals Case of Primary Breast and Ipsilateral Axillary T-Cell Lymphoma: a Rare Occurrence

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Faryal Afridi ◽  
Garry D. Ruben ◽  
Eric Oristian
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
T Cell Lymphomas ◽  
Lost To Follow Up ◽  
Ipsilateral Axilla ◽  
Rare Diagnosis ◽  
Breast Malignancies

Background. Malignant lymphomas of the breast are rare and can be primary or secondary. Non-Hodgkin Lymphoma involving the breast is even rarer comprising 0.04-0.5% of all breast malignancies (Takemura). The incidence is even lower for T-cell lymphomas compared with B-cell subtype. We report the rare incidence of primary T-cell lymphoma involving both breast and ipsilateral axilla. Case. This is the case of an 80-year-old female who initially presented with asymmetry of her right breast. Initial mammograms were inconclusive. MRI could not be performed due to the patient’s severe claustrophobia. The patient was then lost to follow-up but re-presented with a new palpable density in the same breast. Subsequent mammogram showed a suspicious lesion with suspicious right axillary lymphadenopathy. Core biopsy was consistent with T-cell lymphoproliferative disorder involving both the breast and the axilla. She was then referred to medical oncology for management. Conclusion. Although rare, lymphoproliferative disorders of the breast can be encountered during workup for suspicious breast lesions. It is imperative that the surgeon is aware of this rare diagnosis to facilitate appropriate therapeutic intervention.

Alemtuzumab in Combination with CHOP and ESHAP as First-Line Treatment in Peripheral T-Cell Lymphoma.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4740-4740 ◽  
Author(s):  
Tanin Intragumtornchai ◽  
Udomsak Bunworasate ◽  
Thanyaphong Na Nakorn ◽  
Ponlapat Rojnuckarin
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Pneumocystis Carinii ◽  
Elevated Serum ◽  
T Cell Lymphoma ◽  
Newly Diagnosed ◽  
Bulky Disease ◽  
T Cell Lymphomas ◽  
Hodgkin's Lymphomas ◽  
Cmv Disease

Abstract Patients diagnosed with peripheral T-cell lymphomas (PTCL) generally had a poorer prognosis compared to B-cell non-Hodgkin’s lymphomas. With conventional treatment, the 5-year overall and failure-free survivals (OS and FFS) were 36% and 23%, respectively (Vose et al, Blood2005;106:abstract 811). Between February 2005 and January 2006, 13 consecutive patients newly diagnosed with PTCL (5, extranodal nasal NK/T-cell lymphoma, 4 subcutaneous panniculitis-like, 3 PTCL, unspecified and 1 enteropathy type) were enrolled. The median age was 44 years (range, 21–56) and male:female was 1.6:1. Fifty-four percent had stage III/IV, 31%, PS 2–3, 69%, B-symptoms, 15%, bulky disease, 46%, &gt; 1 extranodal site, 38%, elevated serum LDH and 39%, aaIPI 2–3. Twenty-three percent had thrombocytopenia. Patients were treated with alemtuzumab 30 mg. sc. D1-3 of cycle 1–5 plus CHOP (day 1 of cycle 1, 3, 5) and ESHAP (day 1 of cycle 2, 4, 6) at 28-day intervals. Valacyclovir 500 mg tid and trimethoprim/sulfamethoxazole were given for prophylaxis of CMV and Pneumocystis carinii infection, respectively. Of the evaluable 10 patients, complete remission was obtained in 8 patients, 1 had partial remission and 1 had CNS progression while on treatment. Infection was a major adverse complication: 54% had CMV reactivation (1 had CMV disease), 54%, febrile neutropenia and 15%, tuberculosis. With a median follow-up time of 8 months, the 2-year OS and FFS were 75% (95%CI, 41–92) and 48% (95%CI, 14–76), respectively. From the standpoint of this result, alemtuzumab in combination with CHOP and ESHAP is an effective front-line therapy for patients newly diagnosed with PTCL.

Retrospective Study on T Cell Malignent Lymphoma: Classification, Manifestation, Laboratory Finding and Survival.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4652-4652
Author(s):  
Hongyan Tong ◽  
Feng Xiao ◽  
Tieying Dai ◽  
Jie Jin ◽  
Haitao Meng ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
Autologous Bone Marrow Transplantation ◽  
T Cell Lymphoma ◽  
World Health ◽  
Clinical Staging ◽  
T Cell Lymphomas

Abstract T-cell lymphoma is the special malignant type of non-Hodgkin’s lymphoma. The diagnosis and the treatment were usually troublesome for physician in clinical practice. We retrospectively reviewed 63 cases of T-cell lymphomas from 360 cases of lymphomas in our hospital during the period from January 2000 to July 2006. This study is to determine the clinicopathological characteristics of T cell lymphomas. The patients were reclassified according to the World Health Organization classification system. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized. The median follow-up duration was 5 months (range 21days to 36 months). There were slightly more males than females (36 versus 27), and the median age at the onset were 40 years (range 13 to 77 years). The major subtype was peripheral T-cell lymphoma, which accounted for 78% (49/63). Besides, there were 5 cases of anaplastic T large cell lymphoma, 3 lymphoblastic lymphoma, 2 T/NK-cell lymphoma, 2 angioimmunoblastic lymphoma, 1 mycosis fungoides and 1 pre-T cell lymphoma. The most common manifestation was fever, which accounted for 60% (38/63). 27% (17/63) patients presented with obvious enlargement of lymphonodes. Other manifestation included skin rash or phymata, pruritus, jaundice, abdominal pain, rhinorrhagia, puffiness, diarrhea, hoarseness and ulcus. Interestingly, we found that only 32% obvious enlarged lymphonodes could be confirmed by physical examination, hepatomegaly 33% and Splenomegaly 44% respectively. Besides, there were several significant laboratory findings: 40% cases had cytopenia of at least 2 cell lines, 68% had high level of LDH, 70% had elevated β2-microglobulin and 68% were detected T-cell receptor (TCR) and immunoglobulin heavy chain (IgH) gene rearrangement. Furthermore, 53% (33/63) patients had bone marrow involvement at the onset and 27% were diagnosed only by bone marrow biopsy. We also observed 20 cases of lymphoma associated hemophagocytic syndromes (LAHS). The median age for this disease was 37 year. The median life span was 39 days (range 21days to 10 months). The initial manifestations included fever (19/20), splenohepatomegaly (18/20), and cytopenias in all patients. Only 15% patients had enlargement of lymphonodes, which was suggested to be infrequent in LAHS. Immatural T-cell infiltration in bone marrow was detected in 75% (15/20) cases. Chromosome disorder of [der(21)(p11), −22] was detected in 3 cases. We also found that 2 cases which underwent plasmapheresis got much better after chemotherapy. 19 cases were under our follow-up. 17 patients could not survival longer than 6 months. The 6-month overall survival (OS) for LAHS was merely 2 of all 20. Furthermore, nobody survived more than 1 year, which indicated the poor prognosis of LAHS. There were 11 out of 63 cases had received trial chemotherapy including liposomal Doxorubicin, L-asparaginase, velcade, autologous bone marrow transplantation, or plasmapheresis before chemotherapy. The median survival time prolonged obviously from 2 months up to 8 months, which suggested the encouraging efficiency of these methods.

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