T-cell lymphomas: immunologic, histologic, clinical, and therapeutic analysis of 63 cases.

1988 ◽  
Vol 6 (10) ◽  
pp. 1584-1589 ◽  
Author(s):  
B Coiffier ◽  
F Berger ◽  
P A Bryon ◽  
J P Magaud
Keyword(s):  
T Cell ◽  
Clinical Presentation ◽  
Performance Status ◽  
Stage Iv ◽  
Poor Performance ◽  
Large Cell ◽  
Response To Therapy ◽  
Poor Performance Status ◽  
T Cell Lymphomas ◽  
Stage Iv Disease

Sixty-three patients with T-cell lymphoma (TCL) were analyzed to correlate morphological and immunological features with clinical presentation, response to therapy, and survival. Clinical presentation was severe, with 59% of patients having stage IV disease, 60% B symptoms, 35% poor performance status, 44% large tumoral mass, and 40% a high number of extranodal localizations. Morphological subtypes were small-cell in four cases, diffuse-mixed in 29 cases, monomorphic medium-sized in two cases, immunoblastic in 21 cases, anaplastic large-cell in four cases, and unclassified in three cases. Immunological phenotypes were immature T in 11 cases, CD4 in 26 cases, CD8 in 13 cases, and undefined (CD4 + CD8) in ten cases. Response to therapy was poor except for the 39 patients treated by an intensive and sequential regimen (non-Hodgkin's lymphoma [LNH]-80 or LNH-84) that gave a 77% complete remission (CR) rate with a 23% relapse rate. Median survival was 35 months. No correlation was found between morphological subtypes and other variables. Helper (CD4) phenotype seemed to have a better prognosis than other phenotypes. Variables associated with long survival for all the patients were localized disease and absence of large tumoral mass and for the subgroup of patients treated by the LNH regimens CD4 phenotype, absence of B symptoms, absence of a large tumoral mass, and less than two extranodal sites of disease.

Patients with Mature T-Cell Lymphoma Show High Relapse Rates after High Dose Therapy and Autologous Stem Cell Transplantation

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 774-774 ◽  
Author(s):  
Maike Nickelsen ◽  
Carmen Canals ◽  
Norbert Schmitz ◽  
Charalampia Kyriakou ◽  
Monika Engelhardt ◽  
...  
Keyword(s):  
Relative Risk ◽  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Poor Performance ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Poor Performance Status ◽  
High Dose ◽  
Refractory Disease

Abstract Patients (pts) with mature (peripheral) T-cell lymphoma are known to have a poor prognosis when receiving standard conventional chemotherapy. This leads many physicians to regard high dose chemotherapy (HDT) followed by autologous stem cell transplantation (ASCT) as standard therapy for these pts. We here present a retrospective analysis on 424 pts with mature T-cell lymphoma who have received HDT and ASCT in EBMT centres between 2000 and 2005. Patients with primary cutaneous or immature (lymphoblastic) lymphoma were not included. Histological subtypes were anaplastic large cell lymphoma (ALCL, n=98, 23.1%; 19 anaplastic large cell kinase (ALK) positive, 42 ALK negative and for 37 pts the ALK status was unknown), peripheral T-cell lymphoma (PTCLu, n=176, 41.5%), angioimmunoblastic T-cell lymphoma (n=120, 28.3%) and aggressive T-cell lymphoma unspecified (n=30, 7.1%). Median age at ASCT was 51 years (17.2–73.5), median time from diagnosis to ASCT was 9 months (4–99), and median follow up for surviving pts was 36 months (0.4–99). 268 pts (63%) were male. 1/3 received HDT and ASCT after only one previous treatment line. 35% of the pts were treated in CR1, and 52% in chemosensitive disease worse than CR1. At the time point of ASCT only 12 pts (2.8%) had a poor performance status. 9% of the pts received total body irradiation as part of the conditioning regimen. At 3 years after ASCT non relapse mortality (NRM) was 7.4% and the relapse rate (RR) was 43.1%. In multivariate COX analysis for NRM refractory disease (p<0.001, relative risk 6.7) and poor performance status at ASCT (p=0.02, relative risk 3.2) were statistically significant adverse factors while the RR was significantly influenced by histology (PTCLu versus other subgroups, p=0.02, relative risk 1.4), and disease status at ASCT (refractory disease versus CR1 p=0.001, relative risk 3.3 and chemosensitive disease versus CR1 p=0.001, relative risk 1.9). At 3 years progression free survival (PFS) was 49.5% and overall survival (OS) 62.3%. In multivariate COX analysis adjusted for PFS refractory disease and chemosensitive disease worse than CR1 were significant adverse factors compared to CR1 (p<0.001 each, relative risk 3.2 and 1.7, respectively) as was refractory disease compared to chemosensitive disease (including CR1; p=0.004, relative risk 1.9). Other significant adverse factors were age at SCT 60 years (p=0.04, relative risk 1.4), poor performance status at ASCT (p=0.046, relative risk 2.1) and PTCLu versus other subgroups (p=0.02, relative risk 1.4). In summary there was a high RR for this group of pts who actually received ASCT. Especially refractory pts and pts in poor performance status did not benefit from the procedure. Further studies are necessary to better define the pts who will actually be cured by ASCT and to early identify the pts who will need other approaches and thus can be spared high dose chemotherapy which may negatively influence later salvage regimen.

Effective treatment of small-noncleaved-cell lymphoma with high-intensity, brief-duration chemotherapy.

1991 ◽  
Vol 9 (6) ◽  
pp. 941-946 ◽  
Author(s):  
M L McMaster ◽  
J P Greer ◽  
F A Greco ◽  
D H Johnson ◽  
S N Wolff ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Performance Status ◽  
Disease Free Survival ◽  
Poor Performance ◽  
Response To Therapy ◽  
Poor Performance Status ◽  
Inpatient Setting ◽  
Term Survival ◽  
Chemotherapeutic Regimen ◽  
Disease Free

Small-noncleaved-cell (SNC) lymphoma is a high-grade, biologically aggressive neoplasm notable for poor response to therapy, high relapse rate, and less than a 20% long-term survival. We treated 20 patients with SNC lymphoma with a novel chemotherapeutic regimen using intensive doses of chemotherapy at frequent intervals in the inpatient setting. All patients were previously untreated. Sixteen patients (80%) had stage IV disease. Most patients (95%) had at least one other characteristic associated with poor prognosis (bulky [greater than 10 cm] disease, multiple extranodal sites, poor performance status), and 85% had two or more characteristics associated with poor prognosis. Seventeen patients (85%) achieved a complete response (CR) to therapy, including all three patients with human immunodeficiency virus (HIV)-associated disease. There have been three relapses, all occurring less than 18 months after treatment, and two of three relapses occurred in patients who were unable to complete therapy. At a median follow-up of 29 months, 13 patients (65%) remain disease-free; the calculated 5-year actuarial disease-free survival is 60%. Toxicity, chiefly myelosuppression, was severe but manageable. There were two treatment-related deaths, both in elderly patients with poor performance status and advanced-stage disease. These data suggest that such a dose-intensive approach improves the response and survival of patients with SNC lymphoma.

Alemtuzumab in B-Cell Prolymphoytic Leukemia (B-PLL) and Richter’s Transformation.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4733-4733
Author(s):  
Michael Fiegl ◽  
Andreas Falkner ◽  
Michael Fidrik ◽  
Gerhard Postner ◽  
Alois Lang ◽  
...  
Keyword(s):  
Performance Status ◽  
Objective Response ◽  
Clinical Signs ◽  
Stage Iv ◽  
Early Death ◽  
Poor Performance ◽  
Therapeutic Benefit ◽  
Poor Performance Status ◽  
Richter’S Transformation ◽  
Richter's Transformation

Abstract Alemtuzumab is the standard therapy for treatment of patients with relapsed/refractory B-CLL. Significant responses have also been documented in the front-line CLL setting, and as well as in first-line therapy of patients with T-PLL, a population with an exceptionally poor prognosis and few available therapeutic options. Limited data available on the therapeutic benefit of alemtuzumab in other aggressive lymphomas such as B-PLL, which is rare, with a heterogeneous clinical course, and often chemoresistant, as well as CLL with Richter’s transformation, which is also characterized by a poor clinical outcome. Here, we present data on safety and efficacy of alemtuzumab in 6 patients with B-PLL, and 2 cases of B-CLL with Richter’s transformation, both of which developed into DLBCL; one B-CLL case was atypical due to negative CD5 expression (CD19+, CD5−, CD23−). Median age for all 8 patients was 62 years (range, 58–72 years), 5 were male, and all had received a median of 3.5 prior therapies (range, 0–11). Two patients were Rai stage II and III, respectively, and 4 had Rai stage IV disease. At baseline, 3 of the 6 patients with B-PLL had poor performance status, as evidenced by exceptionally high leukocyte counts with clinical signs of hyperleukocytosis and fever of unclear origin. IV alemtuzumab 30 mg was administered according to guidelines (3 times a week, 12 weeks scheduled), but in the majority of cases, dosing was individualized. The median duration of therapy was 4.5 weeks (range, 1–12 weeks), and the median dose was 348 mg (range, 3–793 mg). Therapeutic response was determined according to NCI-WG criteria. In the 2 of 3 patients with poor PS at initiation of therapy an objective response could not be determined due to an early death (septicemia with staphylococcus); 1 patient died prior to achieving a full response due to a suspected apoplectic insult, and another patient died due to progressive disease, shortly after starting alemtuzumab. However, 2 (33%) patients with B-PLL achieved stable disease, lasting 7 months in both cases. Both patients gained a clear clinical benefit from treatment with alemtuzumab as evidenced by CR and PR in peripheral blood, individually, and transfusion independence in both patients. These 2 patients appeared to have favourable disease characteristics, as has been described by other investigators (Leukemia & Lymphoma1999; 33:169), and were diagnosed 9 and 11 years before alemtuzumab, respectively. In the 6 B-PLL patients, overall survival (OS) after start of alemtuzumab therapy was very heterogeneous (0.1; 0.2; 0.3; 1; 27+; 28 months), with a longer OS in the 2 patients with SD. In the 2 patients with B-CLL/Richters’s syndrome (as multifocal DLBCL), PD was observed in one after 2 months on alemtuzumab (survival 15 months). The patient with atypical features was receiving alemtuzumab as an 8th line of therapy and achieved a PR lasting for 13 months (OS 31+ months). In summary, we are adding evidence of therapeutic efficacy of alemtuzumab in a subset of patients with rare, chemotherapy refractory B-lymphoproliferative diseases.

Cytokine/Chemokine Expression with Protein Arrays Identifies Three Subsets of T-Cell Lymphomas with Differented Immunological Profiles,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3473-3473
Author(s):  
Santiago Mercadal ◽  
Antonio Martinez ◽  
Lluis Colomo ◽  
Elias Campo ◽  
Armando Lopez-Guillermo
Keyword(s):  
T Cell ◽  
Conflicts Of Interest ◽  
Expression Profiles ◽  
Stage Iv ◽  
Large Cell ◽  
Protein Microarrays ◽  
High Expression ◽  
Antibody Array ◽  
Image Analysis System ◽  
T Cell Lymphomas

Abstract Abstract 3473 Background: Only a few studies have addressed the expression of cytokine/chemokine in T-cell lymphomas. The aim of this study was to analyze the cytokine/chemokine protein expression profiles and to correlate them with the pathological manifestation of T-cell lymphomas. Patients and methods: Samples at diagnosis from 19 patients were T-cell lymphoma (unspecified peripheral T-cell lymphomas (PTCL), 10 cases; angioimmunoblastic (AITL), 4; ALK-negative anaplastic large-cell (ALCL), 3 and ALK-positive ALCL, 2) and were studied by protein microarrays. The characteristics of the patients were as follows: 11/8 M/F; median age, 55 years. 50% of patients were stage IV, 69% had extranodal involvement, 79% high LDH and 66% high β2 microglobulin. The high or high-intermediate risk IPI and PIT score were 62% and 61%, respectively. We have analyzed the expression of 174 cytokines by using a highly sensitive and specific antibody array containing single band specific primary antibodies duplicate. Chemolumiscence detection was performed by a HRP-conjugated streptavidin in an image analysis system. Densitometry analysis of unsaturated pixels was performed with Image Gauge software and an endogenous housekeeping gene was used to normalize results. Unsupervised clustering analysis was then performed. Results: Three clusters were obtained. Cluster 1 included 5 ALCL, 2 PTCL and 1 AITL, with a cytokine/chemokine profile that had high expression of IL21R, MIG, PDGF AA/AB, LIGHT, IL10, MIP1γ, MIP3α and IL13. Cluster 2 was composed by 5 PTCL and 1 AITL and exhibited a profile characterized by the high expression of L-SELECTIN, MMP1, IL1RII, SIGLEC 5, IL10R, IL1β, IL18BPA, FGF7, IL13RAα2, GMCSF, SDF1β, LIF, IL1Rα and IL11. Finally, the analysis defined a cluster 3 constituted by 2 AITL and 3 PTCL with a cytokine/chemokine profile that showed the high expression of TIMP1, NT4, ITAC and IGBP6. Detailed initial features and outcome of the patients according to the clusters are detailed in the table. Cluster 1 had better survival than the other groups (p=0.01). Summary: T-cell lymphomas show three different cytokine/chemokine patterns as was determined by protein microarrays. Cluster 1 was well characterized with the presence of all ALCL and better survival. Disclosures: No relevant conflicts of interest to declare.

The quantification of the catalytic subunit of telomerase in plasma is a prognostic factor in advanced non-small cell lung cancer (NSCLC) patients

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 7052-7052
Author(s):  
R. Sirera ◽  
C. Camps ◽  
L. Llobat ◽  
A. Berrocal ◽  
R. M. Bremnes ◽  
...  
Keyword(s):  
Advanced Nsclc ◽  
Performance Status ◽  
Stage Iv ◽  
Large Cell ◽  
Complete Response ◽  
Response To Therapy ◽  
Catalytic Subunit ◽  
Stage Iiib ◽  
Circulating Dna ◽  
Nsclc Patients

7052 Background: Qualitative and quantitative analysis of circulating DNA in blood is a promising non-invasive diagnostic and prognostic tool. Our aim was to study the association between the free amount in plasma of the catalytic subunit of telomerase (hTERT) and several clinical variables in advanced NSCLC patients. Methods: We examined 451 NSCLC patients in stage IIIB and IV, treated with cisplatin and docetaxel. Blood samples were collected before chemotherapy, and circulating DNA was extracted from the serum using commercial adsorption columns. The amount of free hTERT in plasma was quantified by using RT-PCR. Results: Median age was 61 years [35–82] and 84% were males. 99% had performance status 0–1. 84% were in stage IV and 16% in stage IIIB. The histological subtypes were: 32% squamous cell carcinoma, 50% adenocarcinoma, 14% anaplastic large cell, and 4% undifferentiated. 41% of the patients received second line chemotherapy. 1% achieved complete response (CR), 36% partial response (PR), 35% had stable disease (SD) and 28% progressive disease (PD). Median hTERT value was 4856 ng/ml; for patients in IIIB was 4847 ng/ml [263–964826] and 4886 ng/ml [67–4373520] in stage IV (p = 0.75). There was not association between hTERT values and response to therapy, 20588 ng/ml [122–317251] in the CR+PR group vs 50204 ng/ml [67–4373520] in the SD+PD group (p = 0.09). hTERT values were not related with the localization of the metastasis. Dividing the cohort in two sets according to hTERT median we found two significantly different groups in terms of Overall Survival (OS) and Time To Progression (TTP). Patients with hTERT <4856 ng/ml had a median TTP of 5.3 months (m) [4.4–6.1] while for hTERT >4856 ng/ml was 4.1 m [3.5–4.6], (p = 0.0009). OS when hTERT <4856 ng/ml was 10.1m [4.9–11.3] and for hTERT >4856 ng/ml was 8.4 m [7.2–9.5], (p = 0.01). In the multivariate analysis, hTERT was an independent predictive variable for TTP (HR 1.39, CI 95% 1.1–1.7, p = 0.002) and OS (HR 1.27, CI 95% 1.1–1.6, p = 0.04). Conclusions: In advanced NSCLC patients, the quantification of free circulating hTERT in plasma is an affordable and valuable prognostic marker. High plasma hTERT levels are a poor prognostic indicator for TTP and OS. No significant financial relationships to disclose.

High correspondence between EGFR mutations in tissue and in circulating DNA form non-small-cell lung cancer (NSCLC) patients (p) with poor performance status (PS)

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 7505-7505 ◽  
Author(s):  
T. Moran ◽  
L. Paz-Ares ◽  
D. Isla ◽  
M. Cobo ◽  
B. Massuti ◽  
...  
Keyword(s):  
Tumor Tissue ◽  
Performance Status ◽  
Stage Iv ◽  
Poor Performance ◽  
Egfr Mutations ◽  
Taqman Assay ◽  
Poor Performance Status ◽  
Nsclc Tissue ◽  
Exon 19 Deletion ◽  
Exon 19

7505 Background: We evaluated the correspondence between EGFR mutations in NSCLC tissue and matched serum DNA and the value of EGFR mutations as a serological marker. Methods: 121 Spanish stage IV NSCLC p received customized first- or second-line erlotinib monotherapy based on the presence of EGFR mutations in the tumor tissue. Serum genomic DNA was obtained from all p prior to erlotinib administration. EGFR exon 19 deletions were studied by length analysis of fluorescently labeled PCR products and the exon 21 L858R mutation by a PCR Taqman assay. Results: The EGFR mutation status in the serum was consistent with that in the tumor tissue of 82/121 p (68%) and of 15/16 p (93.8%) with PS 2 had mutations. Overall, 64.3% of p had an exon 19 deletion and 35.7% had L858R. 78% of mutations were found in females (P=0.01) and 77.6% in never-smokers (P=0.07). Response rate was 88% in p with mutations only in the tumor and 87% in p with mutations in tumor and serum. Complete responses were observed in 20% of p with mutations in tumor and serum vs 4% of p with mutations only in tumor (P=0.09). With a median follow-up of 6.8 months (m) (range, 1.2–17.6), time to progression (TTP) and median survival have not been reached. A trend to better outcome was seen in p without serum EGFR mutations. TTP was longer for p with EGFR exon 19 deletions (not reached) than for p with L858R (7.7 m) (P=0.02). TTP for p with PS 2 with exon 19 deletions was not reached, while it was 2.7 m for p with L858R (P=0.17). Conclusions: EGFR mutations in serum could be a non-invasive source of information on the genotype of the original tumor cells and could be a useful tool to predict p response to erlotinib, especially in p with poor PS. No significant financial relationships to disclose.

Impact of pathologic tumor characteristics in patients with sarcomatoid renal cell carcinoma.

2011 ◽  
Vol 29 (7_suppl) ◽  
pp. 343-343
Author(s):  
B. Shuch ◽  
G. Bratslavsky ◽  
J. H. Shih ◽  
D. Finley ◽  
B. Castor ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Metastatic Disease ◽  
Renal Cell ◽  
Performance Status ◽  
Poor Performance ◽  
Response To Therapy ◽  
Poor Performance Status ◽  
Tumor Characteristics ◽  
Sarcomatoid Renal Cell Carcinoma

343 Background: Patients with sarcomatoid renal cell carcinoma (sRCC) are known to have a poor prognosis and response to therapy. We sought to determine the influence of pathologic tumor characteristics on outcome in order to aid clinical management. Methods: A single center database was reviewed from 1989-2009 to identify all patients with sRCC. Clinical and staging variables were collected and pathologic information including histology, necrosis, percentage of sarcomatoid features (PSF), and microvascular invasion (MVI) was recorded. Influence of clinicopathologic variables on outcome was assessed. Results: A total of 104 patients had confirmed sRCC. The median size of tumors was 9.5 cm (range 2.5-30), 65% of patients had areas of clear cell RCC, and 69.2% had metastatic disease at presentation. The PSF did not influence tumor size, stage, necrosis, MVI, nodes, or metastasis. A total of 85 patients (81.7%) died during the follow-up period with a median survival of 5.9 months. In the overall cohort poor performance status, metastatic disease, and MVI were independent predictors of poor survival. Increased PSF was associated with worse outcome, but it failed to reach significance on multivariate analysis. In a subset analysis of those with non-metastatic disease, MVI and non-clear histology influenced prognosis, but only PSF was the only predictor of outcome. Conclusions: The PSF has limited influence on pathologic characteristics. However, increased PSF amounts may impact survival, especially in those with non-metastatic disease. The presence of MVI is an independent predictor of poor outcome while carcinoma grade and subtype have limited impact on survival. When counseling patients or designing clinical trials for these patients, PSF and MVI, not carcinoma grade or subtype should be considered. No significant financial relationships to disclose.

scholarly journals Nonfollicular small B-cell lymphomas: a heterogeneous group of patients with distinct clinical features and outcome

Blood ◽  
1994 ◽  
Vol 83 (10) ◽  
pp. 2829-2835 ◽  
Author(s):  
F Berger ◽  
P Felman ◽  
A Sonet ◽  
G Salles ◽  
Y Bastion ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Clinical Stage ◽  
Performance Status ◽  
Poor Performance ◽  
Large Cell ◽  
Lactic Dehydrogenase ◽  
Small Cell ◽  
Poor Performance Status ◽  
Advanced Clinical Stage ◽  
Histologic Subtype

Abstract Two hundred sixteen patients with a nonfollicular small cell lymphoma followed up in our department over a 5-year period have been reviewed to define the clinical behavior and survival of patients with each histologic subtype. The respective frequencies of major subtypes were: small lymphocytic/lymphoplasmacytoid lymphoma (immunocytoma, SL/LPL), 28%; large cell-rich immunocytoma (LCRI), 7%; mantle cell lymphoma (MCL), 24%; mucosa-associated lymphoid tissue-lymphoma (MALT-L), 20%; other rare subtypes, 6%; and nonclassified or nonreviewed, 14%. The SL/LPL patients and the MALT-L patients had a relatively indolent disease, usually disseminated for SL/LPL and usually localized for MALT- L. Both subtypes have a long time to treatment failure (TTF; median, 48 and 58 months, respectively) and long survival (median, 118 and 98 months, respectively). The LCRI patients or the MCL patients had more aggressive clinical or biologic features and experienced shorter TTF (median, 26 and 14 months, respectively) and shorter survival (median, 55 and 52 months, respectively). None of these histologic subtypes was associated with a significant cure rate. MALT-L patients did relapse regardless of the initial localization or treatment and at a similar rate to the SL/LPL patients. Factors associated with a worse outcome in nonfollicular small cell lymphoma patients are identical to those described in other lymphoma subtypes: advanced clinical stage, poor performance status, high tumor bulk, and high lactic dehydrogenase or beta 2microglobulin levels. For patients with disseminated disease, standard chemotherapy regimens did not allow a long TTF; therefore, new therapeutic strategies must be developed.

International Peripheral T-Cell Lymphoma (PTCL) Clinical and Pathologic Review Project: Poor Outcome by Prognostic Indices and Lack of Efficacy with Anthracyclines.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 811-811 ◽  
Author(s):  
Julie M. Vose ◽  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Clinical Information ◽  
Poor Performance ◽  
T Cell Lymphoma ◽  
Poor Performance Status ◽  
Cell Index ◽  
Nk T Cell ◽  
Consensus Diagnosis

Abstract The clinical and pathologic features of PTCL using the new World Health Organization (WHO) classification are not well defined. Therefore, we identified 1162 patients diagnosed with de novo PTCL or natural killer (NK)/T-cell lymphoma from 1990 to 2002 at 20 sites in North America, Europe, and Asia. To be eligible, patients needed to be previously untreated, have adequate clinical information with follow up, and pathology slides with immunophenotyping available for a central review. Panels of four expert hematopathologists reviewed all the pathologic materials for each case individually and arrived at a consensus diagnosis according to the WHO classification. The clinical information was submitted and centralized in a database along with the pathology data. The pathology review identified the following case distribution: PTCL-unspecified 22.6%, angioimmunoblastic 18.3%, NK/T-cell 11.7%, diagnoses other than a T-cell lymphoma (10.9%), adult T-cell leukemia/lymphoma (10.7%), anaplastic large cell lymphoma (ALCL-ALK pos) 7.3% and ALCL-ALK neg 5.7%, enteropathy type 4.8%, unclassifiable T-cell lymphoma (2.3%), primary cutaneous ALCL (2.0%), hepatosplenic T-cell (1.5%), subcutaneous panniculitis-like (1.0%), other NK/T-cell (0.9%), blastic NK-cell (0.2%), and gamma-delta T-cell (0.1%). The agreement with the consensus diagnosis ranged from 66 – 98%. Overall clinical characteristics for the patients included a median age of 58 years (range 3 – 92 years), 62% male, 68% stage III/IV, 48% with "B" symptoms, 38% non-ambulatory, 11% with a mass &gt; 10 cm, 20% bone marrow involvement, 31% extranodal disease, 48% with a lactic dehydrogenase (LDH) &gt; normal. Using the standard International Prognostic Index (IPI), 19% had IPI 0/1, 53% IPI 2/3, and 28% IPI 4/5. For all 1162 patients, the 5-year failure-free survival (FFS) is 23% and the 5-year overall survival (OS) is 36%. A multivariate analysis of the two most common types, PTCL-unspecified and angioimmunoblastic, demonstrated the following factors to predict poor OS [poor performance status (p = 0.01), age &gt; 60 years (p = 0.02), platelets &lt; 150K (p&lt; 0.001)]. Factors associated with worse FFS included poor performance status (p &lt; 0.01) and platelets &lt; 150K (p &lt; 0.01). Unlike diffuse large B-cell lymphoma, there was no difference in overall survival comparing patients who did and did not receive an anthracycline for any subtype of PTCL. Comparison of the standard IPI, the T-cell Index (Blood103: 2474–2479, 2004), and our new International T-cell Index on this population demonstrates that in all 3 indices, the FFS and OS are very poor for all patients other than in those with 0 or 0/1 risk factors. Comparison of Prognostic Indices PTCL + Angioimmunoblastic Risk Factors 5-yr FFS 5-yr OS Standard IPI 0/1 32% 49% 2 14% 31% 3 12% 17% 4/5 12% 21% p &lt; 0.001 p &lt; 0.001 Published T-cell Index 0 37% 53% 1 17% 33% 2 11% 18% 3/4 15% 25% p &lt; 0.001 p&lt; 0.001 Current International T-cell Index 0 21% 42% 1 19% 27% 2 10% 19% 3 5% 12% p &lt; 0.001 p &lt; 0.001 In conclusion, the prognosis of most subtypes of PTCL and NK/T-cell lymphoma is poor with standard lymphoma therapies. Novel agents and combinations are needed for improvement in the clinical outcome of these patients.

scholarly journals Peripheral T-cell lymphoma, NOS, and anaplastic large cell lymphoma

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 550-558 ◽  
Author(s):  
Anne W. Beaven ◽  
Louis F. Diehl
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Brentuximab Vedotin ◽  
Response To Therapy ◽  
Anaplastic Lymphoma ◽  
T Cell Lymphomas ◽  
First Remission ◽  
Large Cell Lymphoma

AbstractPeripheral T-cell lymphomas (PTCL), with the exception of anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL), have a very poor prognosis. Although current first line chemotherapy continues to be a CHOP-like (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen there is now data suggesting that the addition of etoposide in younger patients improves outcomes. Even for those patients who do have a response to therapy, the risk of relapse remains quite high. Although autologous transplant in first remission is often used, its role as consolidation therapy in first remission remains unclear and may preferentially benefit low-risk patients. In the relapsed setting, major advances have occurred with Food and Drug Administration (FDA) approval of 4 new agents (pralatrexate, romidepsin, belinostat, brentuximab vedotin) for relapsed/refractory PTCL since 2009. These 4 drugs represent the first agents ever approved specifically for this indication. Unfortunately, with the exception of ALCL for which brentuximab vedotin will likely substantially change our approach to treatment, there are still many patients for whom available drugs will not be effective, and it is for these patients that further advances are urgently needed.

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