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.

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.

Long Term Results of Autologous Hematopoietic Cell Transplantation (AHCT) for Peripheral T Cell Lymphoma: The Stanford Experience.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1906-1906 ◽  
Author(s):  
Andy I. Chen ◽  
Alex McMillan ◽  
Robert S. Negrin ◽  
Sandra J. Horning ◽  
Ginna G. Laport
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Long Term Results ◽  
High Dose ◽  
Refractory Disease ◽  
Anaplastic Lymphoma ◽  
First Remission

Abstract The peripheral T cell lymphomas (PTCL) are uncommon malignancies that typically carry a worse prognosis than the B cell non-Hodgkins lymphomas. There is no uniform standard therapy for PTCL, and AHCT is often offered at relapse or as consolidation in first remission due to the inherently poor prognoses. We conducted a retrospective review of patients who underwent AHCT for PTCL from 1989 to 2006 at Stanford, excluding mycosis fungoides and lymphoblastic lymphoma. Fifty-eight cases were identified: systemic anaplastic large cell (ALCL, n=18), PTCL unspecified (NOS, n=17), angioimmunoblastic (AITL, n=9), nasal type extranodal NK/T (nNK/T, n=7), primary cutaneous anaplastic large cell (n=5), hepatosplenic (n=2), and adult T cell lymphoma/leukemia (n=1). The median age at AHCT was 45 years (range 18–73), and 57% were male. The graft source was mobilized peripheral blood HC in 86% and bone marrow in 14%. The graft was T cell purged in 86% of cases. The conditioning regimen was high dose cyclophosphamide, carmustine, and etoposide in 83% of cases with the rest receiving total body irradiation, carmustine, and etoposide. Fifteen patients were transplanted in first complete or partial response (CR/PR1); 32 in second or beyond CR or PR (CR/PR2+); and 11 with primary refractory disease (REF). The PTCL subtypes transplanted in CR1 (n=12) and PR1 (n=3) included nNK/T (5), AITL (4), NOS (2), hepatosplenic (2), and ALCL (2). Multiple regimens were required to achieve first response in 5 patients. Only one ALK+ (anaplastic lymphoma kinase) ALCL pt was transplanted in CR/PR1 and required 3 induction regimens. The median follow up was 5 years (yr) (range 1–12). For all subjects, five yr overall survival (OS) was 49% and 5 yr progression free survival (PFS) was 24%. Disease status at AHCT had a strong impact on PFS (p=.004) and OS (p=0.06). Five yr PFS was 51%, 17%, and 0%, respectively, and the PFS curve appeared to plateau at 5 yrs. Corresponding OS at 5 yrs for the CR/PR1, CR/PR2+, and REF patients were 76%, 41%, and 36%, respectively. The number of prior regimens and achievement of CR at day +90 post AHCT were also significant factors for PFS by univariate analysis. Age, bone marrow involvement, B symptoms, stage, and histology were not significant for PFS or OS. By multivariate analysis, CR at day +90 remained a highly significant factor for both PFS and OS (p&lt;0.0001 and p=0.0025, respectively). Only 1 patient died from treatment related mortality (&lt;2%). Of interest, 8 patients who progressed after AHCT had a stable CR to subsequent salvage therapy or allogeneic transplant. In summary, about half of PTCL transplanted in CR/PR1 are progression free at 5 yrs, but CR/PR2+ and REF patients fared poorly. CR status at day +90 after AHCT was also a robust predictor for PFS and OS. Based on these results, AHCT may benefit PTCL patients who are consolidated in first remission and deserves further study. In contrast, those with relapsed or refractory disease experienced minimal benefit, and thus novel strategies are especially needed for this group of patients.

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.

Allogeneic Stem Cell Transplantation Results in a Low Relapse Rate in Patients with Peripheral T-Cell Lymphoma.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 974-974
Author(s):  
Maike Nickelsen ◽  
Carmen Canals ◽  
Charalampia Kyriakou ◽  
Norbert Schmitz ◽  
Agnes Buzyn ◽  
...  
Keyword(s):  
Stem Cell ◽  
Relative Risk ◽  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Chronic Gvhd ◽  
T Cell Lymphoma ◽  
Reduced Intensity Conditioning ◽  
Peripheral T Cell Lymphoma ◽  
Reduced Intensity

Abstract Patients (pts) suffering from mature (peripheral) T-cell lymphoma are known to have a poor outcome compared to pts with aggressive B-cell lymphoma when receiving conventional therapies. However, case reports and single centre experiences show promising results of allogeneic stem cell transplantation (allo SCT) for these pts. We here present a retrospective analysis of 103 pts with mature T-cell lymphoma who received an HLA-identical allo SCT in EBMT centres between 2000 and 2005. Histological subtypes of the patients were anaplastic large cell lymphoma (n=25; 4 anaplastic lymphoma kinase [ALK] positive, 11 ALK negative, 10 ALK unknown), peripheral T-cell lymphoma unspecified (n=51), angioimmunoblastic T-cell lymphoma (n=18) and aggressive T-cell lymphoma unspecified (n=9). Patients with primary cutaneous T-cell lymphoma or T-lymphoblastic lymphoma were excluded. Median follow up for surviving patients was 40 months (6–98), median age at allo SCT 42 years (18–68) and median time to allo SCT 17 months (3–233). 39 pts had failed a prior autologous SCT; 68 pts had chemosensitive disease at allo SCT (of these 15 in CR1) and 30 pts had chemorefractory or untested relapsed or progressive disease. Donors were HLA identical siblings for 73 and matched unrelated donors for 30 pts; 82 pts received peripheral blood stem cells. 54 pts were treated with reduced intensity conditioning before SCT. The cumulative incidence (CI) of acute graft versus host disease (GvHD) 100 days after SCT was 52% and had no effect on non-relapse mortality (NRM) or relapse rate (RR). 34 of 74 evaluable pts experienced chronic GvHD (14 limited, 20 extensive), CI 46% at 2 years. Introducing chronic GvHD as a time dependent covariate, this factor was associated with a higher NRM (p<0.001) and a lower RR (statistically not significant), resulting in a lower progression free survival (PFS) (p=0.001). For all patients CI of NRM and RR at 3 years were 36.7% and 24.9%, respectively, resulting in 41.4% PFS and 46.9% OS. Median time to relapse / progression was 4 (1–18) months. In multivariate COX analysis for PFS refractory disease (relative risk 3.4, p=0.001), prior failed auto SCT (relative risk 2.6, p=0.001) and poor performance status at SCT (relative risk 2.5, p=0.04) were significant adverse factors. Additionally, there were trends for T-cell depleted grafts (p=0.1), CMV status other than negative-negative (p=0.1) and reduced intensity conditioning (p=0.2) to be adverse factors. A subgroup of 39 good risk pts (good performance status, chemosensitive disease, no prior autologous SCT) had a PFS of 59%. This retrospective analysis on allo SCT in peripheral T-cell lymphoma shows an encouraging low RR but efforts have to be made to reduce NRM. Prospective studies are warranted to further define pts who will profit from an early allo SCT.

scholarly journals An Update in Management of Noncutaneous T-Cell Lymphomas

2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Y. Y. Hwang ◽  
R. H. S. Liang
Keyword(s):  
T Cell ◽  
Treatment Approach ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
High Dose ◽  
Conventional Chemotherapy ◽  
High Dose Therapy ◽  
T Cell Lymphomas ◽  
Alk Positive

T-cell lymphoma is a heterogeneous group of diseases. Except for ALK positive anaplastic large cell lymphoma, T-cell lymphoma responds to conventional chemotherapy unfavourably, and most patients carry poor prognosis. In recent years, efforts have been made to improve the outcome of T-cell lymphoma patients. Novel agents, high-dose therapy, and allogeneic stem cell transplantation are studied, and various results are reported in literature. This paper looks into the prognostication and treatment approach of different entities of noncutaneous T-cell lymphoma and would focus on the latest updates in its management.

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.

Second-Line Therapy with ICE Followed by High Dose Therapy and Autologous Stem Cell Transplantation for Relapsed/Refractory Peripheral T-Cell Lymphomas: Minimal Benefit When Analyzed by Intent To Treat.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2679-2679 ◽  
Author(s):  
Steven Horwitz ◽  
Craig Moskowitz ◽  
Tarun Kewalramani ◽  
Paul Hamlin ◽  
David Straus ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
High Dose ◽  
Refractory Disease ◽  
Second Line ◽  
High Dose Therapy ◽  
T Cell Lymphomas ◽  
Peripheral T Cell Lymphomas

Abstract Background: Peripheral T-cell lymphomas (PTCL) are a heterogeneous group of non-Hodgkin’s lymphomas with a poorer prognosis than their B-cell counterparts. Second -line chemotherapy (SLT) followed by high dose therapy and autologous stem cell transplantation (HDT/ASCT) is a common approach to patients (pts) with relapsed PTCL. Reports evaluating this approach have analyzed the outcome of transplanted patients. Our goal was to identify pts with relapsed or refractory disease, treated in a uniform manner, with the intent to induce a remission and proceed to HDT/ASCT. Patients and Methods: From our ICE database we identified 40 pts with a diagnosis of a mature T or NK lymphoma who received ICE as SLT therapy prior to planned HDT/ASCT. Histologic subtypes were as follows: PTCL NOS (n=15), anaplastic large cell lymphoma (n=11), angioimmunoblastic T-cell lymphoma (n=5), NK/T cell lymphoma (n=4), subcutaneous panniculitis-like T-cell lymphoma (n=2), and one each of hepatosplenic γ/δ T-cell lymphoma, enteropathy associated T-cell lymphoma, and transformed mycosis fungoides. Median age was 48 years (24–73), five pts were ≥ 60 years. Twenty-five men and 15 women were treated. Initial chemotherapy regimens included: CHOP/CHOP-like (n=32), NHL-15 (n=5), and other (n=4). Twenty-two pts responded to their initial therapy and 18 had primary refractory disease. At time of relapse, 24 pts had stage IV disease, 23 had elevated LDH, and 9 had a performance status ≤ 70%. Thirty-one pts had at least one extranodal site. Complete second line International Prognostic Index (IPI) factors (AA stage, LDH, PS) were available for 36 pts. IPI scores were LR (n=3), LIR (n=12), HIR (n=13), and HR (n=8). Results: All pts received at least one cycle of ICE, 36 received all three planned cycles. Fourteen patients (35%) achieved a complete response, 14 (35%) had a partial response, four (10%) had stable disease, and eight (20%) progressed on therapy. Twenty-seven (68%) pts went on to receive HDT/ASCT. Median follow-up for surviving pts was 45 months (range 7–104). By 3 years, 33/40 (83%) of pts had relapsed with a median progression free survival (PFS) of 6 months from last ICE treatment. Twenty-eight (70%) pts relapsed within 1 year. Neither second-line IPI nor histologic subtype was predictive of continuous remission. Relapsed pts had a superior 3 year PFS compared to primary refractory pts 20% vs 6% (p=0.0005). Despite high relapse rate, 18/40 (45%) of pts were alive at last follow-up. Conclusion: SLT and HDT/ASCT is a common treatment strategy for pts with relapsed PTCL. However, when examined by intention to treat from initiation of SLT and not from time of transplant few pts have durable benefit from this approach. Despite the high response rate to SLT, consolidation with HDT/ASCT provides little clinically meaningful benefit for most pts. The number of pts alive with other therapies despite early relapse underscores the need to study alternative or maintenance strategies for those who achieve remissions.

Matched Unrelated Donor Transplantation Is Curative In Selected Patients with Diffuse Large B Cell Lymphoma: A Report of the Lymphoma Working Party (LWP) of the European Group for Blood and Marrow Transplantation (EBMT)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 363-363 ◽  
Author(s):  
Irit Avivi ◽  
Carmen Canals ◽  
Jian Jian Luan ◽  
Gerald. G. Wulf ◽  
Arnon Nagler ◽  
...  
Keyword(s):  
Stem Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Conditioning Regimen ◽  
Poor Performance ◽  
Poor Performance Status ◽  
Unrelated Donor ◽  
Refractory Disease ◽  
Matched Unrelated Donor ◽  
Matched Sibling

Abstract Abstract 363 Matched unrelated donor stem cell transplantation (MUD-SCT) has recently become a therapeutic option for patients with B cell diffuse large cell lymphoma (DLBCL) who fail other conventional therapies and do not have a matched sibling donor available. We present retrospective data of 473 DLBCL patients, 285 males and 188 females, aged 18 to 69 years (median 46 years), treated with matched sibling (n=301) or matched unrelated (n=172) SCT between January 2000 and December 2007 and reported to the EBMT registry. Median time from diagnosis to allograft was 25 months (range 2 – 203). Fifty-seven percent of the patients had failed previous autologous SCT, and 12% were transplanted in chemorefractory disease. After a median follow up of 41 months, the estimated 3-year non-relapse mortality (NRM), relapse rate (RR), progression free survival (PFS) and overall survival (OS) for the whole series were 37%, 32%, 31% and 40%, respectively. There were no statistically significant differences in patient age, disease status at transplantation, stem cell source and intensity of conditioning regimen between the Sib and the MUD cohorts (summarized in Table 1).However, a higher number of patients undergoing MUD have already failed an autograft (66% vs 52%, p=0.004), and T cell depleted grafts were more frequently used in the MUD cohort. Acute GvHD occurred in 48% of patients treated with a sib allograft vs 54% in those receiving a MUD (p=n.s). Interestingly, there were no statistically significant differences in NRM and RR between the Sib and the MUD groups (3 yr NRM=38% vs 37%, RR approached 38% vs 34%), resulting in a similar 3-year PFS and OS (32% vs 29% and 42% vs 35%, respectively). Multivariate analysis identified refractory disease (p = 0.001, RR 3.2; CI=1.3-3.5), poor performance status (p = 0.017, RR 1.9; CI=1.1-3.4) and age at transplantation > 50 years (p = 0.015, RR1.5; CI=1.1-2.1) as independent adverse prognostic factors for NRM and time from diagnosis to SCT < 36 months (p = 0.02, RR 1.5; CI=1.1-2.1), a previously failed autologous SCT (p = 0.026, RR .4; CI=1.1-1.9), refractory disease (p = 0.003, RR 2; CI=1.3-3.1), poor performance status (p = 0.0001, RR 3.5; CI=2.1-5.6) and T cell depletion (p = 0.001, RR 2; CI=1.3-2.9) as adverse prognostic factors for RR. A previous autologous SCT, poor performance status and refractory disease at transplantation were found to be significantly associated with a shorter PFS (p=0.04, RR 1.3; CI=1-1.6; p=0.0001, RR 2.4; CI=1.6-3.4; p=0.000,RR 2.1; CI=1.5-2.9, respectively) and refractory disease (p=0.0001, RR 2.2; CI=1.6-3.1) and poor performance status at the time of SCT (p=0.0001, RR 2.5; CI=1.7-3.6) with a shorter OS. Patients allografted from MUD tended to have a slightly increased risk of overall mortality in multivariate analysis, but this was not statistically significant (p=0.06, RR 1.2; CI=0.95-1.6). In conclusion, MUD SCT provides a curative option, not significantly inferior to that obtained with a matched sibling transplant, for selected patients with DLBCL who have failed conventional therapies, including autograft. Similarly, the type of conditioning regimen was not found to have a significant impact on patient outcome. Table 1. Clinical characteristics Sib vs MUD Sib (n = 301) MUD (n= 172) p Age (median, years) 46 45 N.S. Previous ASCT 48% 33% 0.004 Refractory disease 12% 12% N.S. Poor P.S. 13% 7% 0.06 RIC/CC 57%/43% 56%/42% N.S. SC source BM/PB 20%/80% 22%/78% N.S. ATG/ALG 15% 46% <0.001 in vivoüT-cellüdepletion 12% 20% 0.03 ex vivoüT-cellüdepletion 5% 12% 0.03 ASCT. Autologous stem cell transplantation; PS. Performance status; RIC. Reduced intensity conditioning; CC. Conventional conditioning; SC. Stem cell; BM. Bone marrow; PB. Peripheral blood; ATG/ALG. Anti-thymocyte globulin/ALG. Anti-lymphocyte globulin. Disclosures: No relevant conflicts of interest to declare.

Enteropathy-Type Intestinal T-Cell Lymphoma: Clinical Features and Treatment of 31 Patients in a Single Center

2000 ◽  
Vol 18 (4) ◽  
pp. 795-795 ◽  
Author(s):  
Joanna Gale ◽  
Peter D. Simmonds ◽  
Graham M. Mead ◽  
John W. Sweetenham ◽  
Dennis H. Wright
Keyword(s):  
Celiac Disease ◽  
T Cell ◽  
Small Bowel ◽  
Clinical Features ◽  
Cell Lymphoma ◽  
Bowel Perforation ◽  
Survival Rates ◽  
T Cell Lymphoma ◽  
Poor Performance Status ◽  
High Dose

PURPOSE: We report the clinical features and treatment of 31 patients with a diagnosis of enteropathy-type intestinal T-cell lymphoma treated at the Wessex Regional Medical Oncology Unit in Southampton between 1979 and 1996 (23 men, eight women). PATIENTS AND METHODS: Patients were identified from our lymphoma database. Details of history, physical examination, staging investigations, treatment, and outcome were taken from patient records. RESULTS: Twelve patients (35%) had a documented clinical history of adult-onset celiac disease, and a further three had histologic features consistent with celiac disease in resected areas of the small bowel not infiltrated with lymphoma. After diagnosis, 24 (77%) of the 31 patients were treated with chemotherapy; the remaining seven had surgical treatment alone. More than half were unable to complete their planned chemotherapy courses, often because of poor nutritional status; 12 patients required enteral or parenteral feeding. A response to initial chemotherapy was observed in 14 patients (complete response, n = 10; partial response, n = 4). Observed complications of treatment were gastrointestinal bleeding, small-bowel perforation, and the development of enterocolic fistulae. Relapses occurred 1 to 60 months from diagnosis in 79% of those who responded to initial therapy. Of the total 31 patients, 26 (84%) have died, all from progressive disease or from complications of the disease and/or its treatment. The actuarial 1- and 5-year survival rates are 38.7% and 19.7%, respectively, with 1- and 5-year failure-free survival rates of 19.4% and 3.2%, respectively. CONCLUSION: The prognosis for these patients is poor. This, in part, reflects late diagnosis and poor performance status at the time of presentation. The role of salvage treatments and high-dose chemotherapy at relapse is not clear. However, it is encouraging that there are five long-term survivors in our patient population.

Angioimmunoblastic T-Cell Lymphoma: A Large Series Outcome Analysis.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2810-2810
Author(s):  
Charalampia Kyriakou ◽  
Ayoma Attygalle ◽  
David Linch ◽  
Anthony Goldstone ◽  
Paul Smith ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
T Cell Lymphoma ◽  
Outcome Analysis ◽  
High Dose ◽  
First Line ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
First Line Therapy ◽  
Line Therapy

Abstract Angioimmunoblastic T-cell Lymphoma (AITL) is a rare Peripheral T-cell lymphoma that primarily affects the elderly, presenting as advanced disease characterized by aggressive behaviour and very poor outcome. Despite the unfavourable prognosis, the best approach for treating patients with AITL is still unknown. We report a retrospective multicentre study of 64 AITL patients (37 male, 27 female) who were diagnosed as AITL in our institution based on typical histology and molecular clonality analysis between 1995 and 2004. The median age at diagnosis was 60 years (range 25 to 87). Fifty-two patients (81%) presented with advanced stage III–IV disease, 41 (64%) had B symptoms and 49 (76%) elevated LDH. ECOG performance status was 1 for 40%, 2 for 53% and 3 for 9% of the patients. Based on IPI risk factors 12% of the patients were classified as low risk, 17% low intermediate, 29% high intermediate and 42% as high risk. Six patients developed autoimmune haemolytic anaemia. Therapeutic approach varied from no treatment to high dose therapy (HDT). The majority of the patients had received CHOP chemotherapy. Overall 19 patients (30%) had received 1 treatment line, 20 (31%) had 2, 19 (30%) 3, 4 patients (6%) were treated with ≥4 and 2 (3%) were not eligible for any treatment. Twenty patients (31%) proceeded to an autologous and 2 to allogeneic transplantation after achieving CR (n=12), PR (n=7) while 3 had progressive disease. Following first line therapy 37 patients (58%) achieved CR, 15 (23%) PR and 10 (16%) had primary refractory disease. Median time to relapse or progression was 6 months (1 to 89). Interestingly three patients relapsed as EBV driven DLBCL, 1as DLBCL and 2 as EBV driven Hodgkin’s lymphoma. With a median follow-up of 19 months (1 to 119) twenty-six patients (41%) were alive. Eighteen (28%) of these patients were in CR, and 8 in PR (13%). Thirty-seven patients (58%) died, 29 (45%) from disease progression. Twelve patients developed toxic complications -infectious complications (n=8), haemorrhage (n=1), thrombosis (n=1) myocardial infarction (n=2). The estimated PFS rates at 1 and 2 years were 33% and 27% respectively. Overall survival rates were estimated at 55% and 28% at 2 and 4 years. By univariate and multivariate analyses, no response to first line therapy (p=0.035) and male sex (p=0.0161), were significantly associated with higher relapse rate. Application of HDT resulted to significantly superior PFS (p=0.002). Poor performance status at presentation was the only factor found to be significant for the OS (p= 0.0317). In conclusion analysis of the results of this large cohort of AITL patients showed that although the initial overall response rate was 73% this was short lived. It is estimated that less than 30% of the patients will survive and remain disease free at 4 years. Considering the dismal outcome with current therapeutic approaches, new strategies using novel agents to improve further and most importantly maintain initial response are needed. The role of frontline HDT either autologous or allogeneic for eligible AITL patients is worth exploring in prospective collaborative studies.

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