Primary Mediastinal Large B-Cell Lymphoma: Treatment Outcomes in 43 Patients.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4754-4754
Author(s):  
Celso L. Mello ◽  
Vladimir C. Lima ◽  
Christiane Valadares ◽  
Soares A. Fernando ◽  
Carvalho A. Lopes ◽  
...  

Abstract Objectives: Primary mediastinal B-cell Lymphoma (PMBL) is recognized as a separate entity in the WHO classification. Treatment for PMBL is based on a combination of conventional dose chemotherapy, high dose chemotherapy and radiation therapy. The best strategy is still undefined. We conducted a retrospective analysis of patients with PBML to identify clinical prognostic factors. Materials and Methods: Between 1989 and 2004, 43 consecutives patients treated at Hospital do Cancer AC Camargo, São Paulo, Brazil were identified and clinical and histological data were reviewed. All patients had previous diagnosis of diffuse large B-cell lymphoma, with positive CD20 on neoplastic cells. For patients with extrathoracic involvement at presentation a predominant anterior mediastinal lesion should be present. Induction chemotherapy regimens were grouped in first generation (CHOP or CHOP-like), third generation (PromaceCytabon or MACOP) and other (pediatric regimens, COP). Results: Age ranged from 16 to 82 years-old, 30 females and 13 male. Patients aged < 35 yo had better outcome compared with those older than 35 yo (5 years OS - 56% x 34%, p=0.048). Among clinical variables, female gender, stage IA-IIB, IPI 0-1, normal LDH, absence of mediastinal bulky disease were associated with better prognosis, although not statistically significant. Response rate to first generation regimens was: 37% CR (11/29), 24% PR (7/29) and 24% PD (7/29). Four patients were treated with third generation regimens with 2 CR and 2 PR. 20 out of 25 patients with PR or CR to first line chemotherapy received mediastinal radiation therapy. More than 65% of patients had a minimum time of follow up 5 years. With a median follow up of 22.3 months, projected 5 year OS was 47% and for the responders the median PFS was 8,4 months. No difference in OS and PFS was observed among the three chemotherapy groups. Conclusion: Our analysis showed that response rate to first line regimens was around 60% and 25% of patients were primarily refractory to CHOP regimen. Age younger than 35 years old was associated with a better prognosis. 5 years overall survival was 45 % and is in accordance with the literature. Although recent studies have demonstrated biological similarities between PMBL and Hodgkin’s Lymphoma, the prognosis of PMBL is less favorable than HL. Better understanding of the disease will help in developing more appropriate therapeutic strategies for PMBL.

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 17556-17556
Author(s):  
C. L. Mello ◽  
O. Feher ◽  
V. C. Lima ◽  
C. Valadares ◽  
F. A. Soares ◽  
...  

17556 Objectives: Primary mediastinal B-cell Lymphoma (PMBL) is recognized as a separate entity in the WHO classification. Treatment for PMBL is based on a combination of conventional dose chemotherapy, high dose chemotherapy and radiation therapy. The best strategy is still undefined. We conducted a retrospective analysis of patients with PBML to identify clinical prognostic factors. Methods: A retrospective analysis of 43 patients treated at Hospital do Cancer AC Camargo, Sao Paulo, Brazil, between 1989 and 2004. All patients had previous diagnosis of diffuse large B-cell lymphoma, with positive CD20 on neoplastic cells. A predominant anterior mediastinal lesion should be present. Induction chemotherapy regimens were grouped in first generation (CHOP/CHOP-like), third generation (PromaceCytabon/MACOP) and other (pediatric regimens, COP). Results: Age ranged from 16 to 82 years-old, 30 females and 13 male. Age < 35 yo was associated with a better prognosis (5 years OS - 56% × 34%, p = 0.048). Among clinical variables, female gender, stage IA-IIB, IPI 0–1, normal LDH, absence of mediastinal bulky disease were associated with better prognosis, although not statistically significant. Response rate to first generation regimens was: 37% CR (11/29), 24% PR (7/29) and 24% PD (7/29). Four patients were treated with Third generation regimens with 2 CR and 2 PR. 20 out of 25 patients with PR or CR to first line chemotherapy received mediastinal radiation therapy. More than 65% of patients had a follow up of 5 years or more. With a median follow up of 22.3 months, projected 5 year OS was 47% and for the responders the median PFS was 8,4 months. No difference in OS and PFS was observed among the three chemotherapy groups. Conclusion: Our analysis showed that response rate to first line regimens was around 60% and 25% of patients were primarily refractory to CHOP regimen. Age younger than 35 years old was associated with a better prognosis. 5 years overall survival was 45% and is in accordance with the literature. Although recent studies have demonstrated biological similarities between PMBL and Hodgkin’s Lymphoma, the prognosis of PMBL is less favorable than HL. Better understanding of the disease will help in developing more appropriate therapeutic strategies for PMBL. No significant financial relationships to disclose.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2770-2770
Author(s):  
Luis Fayad ◽  
Michael Overman ◽  
Barbara Pro ◽  
Peter McLaughlin ◽  
Felipe Samaniego ◽  
...  

Background: Follicular lymphoma grade 3 has a natural history that is more akin to that of diffuse large B-cell lymphoma. The addition of rituximab to standard CHOP has resulted in improved response and survival in diffuse large B-cell lymphoma. Information about outcomes in follicular lymphoma grade 3 is lacking. Methods: A single institution retrospective review of patients with follicular grade 3 lymphoma evaluated at the UTMDACC from 1999 to 2004. Patients were located from the UTMDACC lymphoma database. All patients were initially treated with R-CHOP. Results: Forty-five patients were identified: 51% male, 47% ≥60 years, and 87% follicular grade 3b. The LDH was elevated in 24%, ECOG performance status was >1 in 2%, and >1 site of extranodal involvement was present in 10%. Stage distribution was 11% stage I, 11% stage II, 42% stage III, and 36% stage IV, bulky disease (>7cm) was present in 11%, and B symptoms occurred in 13%. Beta-2 microglobulin was elevated in 57% with values >3 μg/dL in over 50%. IPI distribution was: 46% IPI Low, 38% LI, 11% IH, and 4% IPI High. Overall response rate was 100% with 96% complete responses. Relapse rate by IPI category was 24% for Low IPI, 18% for IPI LI, and 40% for IPI IH, and 100% for the two patients with High IPI. With median follow-up of 33 months, three year failure-free survival (FFS) is 73% (95% CI: 59 to 87%). One patient died (2%) with an overall survival (OS) at three years of 97% (95% CI: 93 to 100%). Conclusion: The addition of rituximab to CHOP provided a high response rate and excellent early survival in this group of mostly good prognosis patients. Relapses were still seen; longer follow-up is needed.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4878-4878
Author(s):  
Heidi Mocikova ◽  
Jozef Michalka ◽  
Jan Koren ◽  
Pavla Stepankova ◽  
Alexander Wild ◽  
...  

Abstract Abstract 4878 Background. Strong CD20 expression in nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) suggests the feasibility of rituximab in the treatment of this disease. Methods. We analysed the outcome of 102 patients with NLPHL treated with or without rituximab in combination with conventional treatment: chemotherapy and/or radiotherapy. Histologies were reviewed for the purpose of this study. Rituximab was administered in 26 of 102 NLPHL patients (13 in the first line treatment and in 13 of 20 relapsed patients). Additionally, rituximab with chemotherapy was administered in 11 patients with histologic transformation to diffuse large-B cell lymphoma. Median follow-up was 7.1 years. Median patient age was 34.2 years. Results. The 10-year overall survival (OS) rate and progression - free survival (PFS) of the whole group was 88% and 65%, respectively. There was no difference in OS and PFS in patients with clinical stage IA without risk factors treated without or with rituximab (30 vs 3 patients) and conventional treatment, however the follow-up in the rituximab group was short. The addition of rituximab to conventional treatment did not affect the OS in the group of patients with more advanced disease: 58 patients without vs 10 with rituximab (94% [95% CI: 88 – 100%] vs 100% [-], P=0.566). PFS in both groups did not differ significantly in the first line treatment (69% [95% CI: 57 – 82%] vs 100% [-], P=0.165), however when all lines of treatment were analysed, PFS was significantly better in patients treated without rituximab (92% [95% CI: 84 – 100%] vs 38% [95% CI: 22 – 65%], P< 0.001). Histologic transformation to diffuse large B - cell lymphoma was diagnosed in 11 rituximab naive patients, but this was not statistically significant when compared to 0 patients after rituximab treatment (14,5% vs 0%, P=0.061). Histologic transformation was the only poor prognostic factor that influenced OS (HR 7.936, P=0.004). Conclusions. Rituximab does not prevent relapses in NLPHL. This study confirms favorable OS of NLPHL patients regardless whether rituximab was used or not. The absence of histologic transformation in NLPHL patients treated with rituximab deserves further investigation. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4440-4440
Author(s):  
Beatrice Casadei ◽  
Alessandro Broccoli ◽  
Vittorio Stefoni ◽  
Cinzia Pellegrini ◽  
Letizia Gandolfi ◽  
...  

Abstract The first line treatment for primary mediastinal large B-cell lymphoma (PMLBCL) still remains a matter of debate even if the literature confirms that an anthracycline-containing regimen should be the main choice. The European experience shows the superiority of “third-generation” dose-dense regimen like MACOP-B (methotrexate, doxurubicin, cyclophosphamide, vincristine, bleomycin, prednisone) or VACOP-B (same as MACOP-B, with etoposide instead of methotrexate) over CHOP (cyclophosphamide, adriamycin, vincristine and prednisone) or CHOP-like regimen. The addition of rituximab to third-generation regimen does not seem to cause any advantages in terms of overall survival (OS) and progression free-survival (PFS), while external radiotherapy (RT) has shown a good efficacy as a consolidation strategy at the end of induction chemotherapy, especially in converting partial responses (PR) into complete responses (CR). Between October 1989 and April 2010, 98 (58 females and 40 males) previously untreated PMLBCL patients were diagnosed and subsequently treated at our Institution. All patients were treated with MACOP-B regimen, concurrent rituximab was administered in 57 patients (58.2%) and 67 patients (68.4%) received mediastinal RT. Among 57 patients who received rituximab, 37 (64.9%) underwent RT whereas, among 41 who did not receive rituximab, RT was delivered in 30 (group 4, 73.2%) patients. 11 patients (group 1, 11.2%) received chemotherapy alone and 37 (group 3, 37.8%) received besides immunotherapy and RT (Table 1). All patients were assessed at the diagnosis and after the treatment with computed tomography and positron emission tomography (after 2001) scan. Main aims of our study were the effectiveness of the regimen measured as overall response rate (ORR) and patients survival. Sixty-one (62.2%) out of 98 patients achieved a CR and 27 (27.6%) were in PR after 12 cycles of MACOP-B regimen (with or without rituximab). Twenty-one patients in PR after (immuno)chemotherapy converted the response into CR with mediastinal RT. At the end of the scheduled treatment, 82 patients (83.7%) achieved a CR and 6 a PR (6.1%), yielding an ORR of 89.8%. At a median follow-up of 5.6 years, 9 patients relapsed within the first 2 years of treatment. During the follow-up 15 patients died, of whom 13 as a consequence of disease relapse or progression. The projected OS at 17 years is 72% with a PFS and a disease free survival (DSF) of 86.8% and 88.4% respectively (Figure 1 A-C). The subgroup analysis shows a statistically significant difference in term of OS (p=0.0003) but not in term of PSF and DFS among the four groups of treatment (Figure 1 D-F). All the patients receiving consolidation RT obtained a CR without differences between subgroup 3 and 4. RT seems to have a small consolidative potential in patients who obtained CR after chemo-immunotherapy alone: there are no differences in term of DFS between subgroup 2 and 3. No statistically significant differences in terms of OS, PFS and DSF occurred among patients received rituximab or not, regardless of a subsequent RT. Our monocentric experience spans a period of 20 years and indicates that a third-generation regimen like MACOP-B is feasible and could be a standard of first line treatment for PMLBCL. In agreement with the literature, adding rituximab doesn’t improve the outcome. Mediastinal RT, delivered as a consolidative strategy, impacts on global survival and on CR rates. In particular, RT after third-generation regimen remains a good strategy to convert PR into CR, but it may be avoided in patients obtaining CR after (immuno)chemotherapy. Table 1 Group MACOP-B Rituximab Radiotherapy N (%) 1 Yes No No 11 (11.2%) 2 Yes Yes No 20 (20.4%) 3 Yes Yes Yes 37 (37.8%) 4 Yes No Yes 30 (30.6%) TOTAL N (%) 98 (100%) 57 (58.2%) 67 (68.4%) 98 (100%) Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3302-3302
Author(s):  
Ute Hegenbart ◽  
Michael Rieger ◽  
Matthias Witzens ◽  
Manfred Hensel ◽  
Anthony D. Ho

Abstract Primary mediastinal large B-cell lymphoma represents a distinct subset of large B cell lymphoma, and it has been shown to occur preferentially in young females with a bulky mediastinal mass. Between 1996 and 2004, a total of 43 patients (pts) with primary mediastinal lymphoma were diagnosed and treated at our institution, 34 of them are evaluable for response and long-term follow-up. Twenty females and 14 males have been treated, the median age at diagnosis was 33 years (range: 18–56), 10 pts had stage I, 19 pts stage II and 5 pts stage III disease, respectively. Fifteen pts presented with B symptoms, LDH was increased in 68% of pts. First-line chemotherapy included CHOP (2 pts), R-CHOP (3 pts), CHOEP (8 pts), R-CHOEP (13 pts) or Mega-CHOEP (8 pts). 16 pts received Rituximab in combination with chemotherapy as front-line therapy. Complete response after first-line therapy has been observed in 71% and PR in 21% of pts. Later on, two pts in CR and 1 pt with PR relapsed after conventional chemotherapy. Radiotherapy was administered in 22 pts (65%) presenting with initial bulky disease. 19 pts have been autografted, 15 of them as part of up-front therapy. Indications for up-front autologous HCT were treatment within the Mega-CHOEP protocol (n=8), PR after first-line chemotherapy (n=3), IPI Score of 2 (n=2) and individual decision in 2 pts. Allogeneic HCT was performed as relapse therapy in 4 patients. Of the 15 pts who had up-front autologous HCT, 13 remained in CR. Overall, thirty pts were alive at the date of last contact with a median follow-up since diagnosis of 25 months (range 3–99). Three of 4 pts who had undergone allogeneic HCT in a advanced phase of disease died of transplant-related complications. In conclusion, in this patient group addition of Rituximab and/or dose-intensified first-line chemotherapy lead to a high rate of complete remissions as compared to historic controls. Whether up-front high-dose chemotherapy with autologous HCT or the administration of radiotherapy to the initial bulk improve PFS and OS remains an open question for this special lymphoma entity. The achievement of complete remission with primary treatment strategies seems to be essential to achieve long-term cure for this disease.


Author(s):  
Richard T Maziarz ◽  
Jie Zhang ◽  
Hongbo Yang ◽  
Xinglei Chai ◽  
Chengbo Yuan ◽  
...  

No head-to-head trials have compared the efficacy of tisagenlecleucel versus historical treatments for adults with relapsed or refractory diffuse large B-cell lymphoma (r/r DLBCL). This study indirectly compared the overall survival (OS) and overall response rate (ORR) associated with tisagenlecleucel, using data from the JULIET study (NCT02445248), versus historical treatments assessed in the CORAL study follow-up population. To assess treatment effects in the treated (full analysis set [FAS]) and enrolled (intent-to-treat [ITT]) study populations, the JULIET FAS vs. the CORAL follow-up FAS and JULIET ITT vs. CORAL follow-up ITT populations were separately compared. Propensity score weighting using standardized mortality ratio weight (SMRW) and fine stratification weight (FSW) was used to compare OS and ORR, adjusting for baseline confounders. The results indicated that tisagenlecleucel was associated with a lower hazard of death among the FAS (adjusted hazard ratio [95% CI], both FSW and SMRW: 0.44 [0.32, 0.59]) and ITT populations (FSW: 0.60 [0.44, 0.77], SMRW: 0.57 [0.44, 0.73]; all p&lt;0.001). Median OS was 12.48 months (JULIET) vs. 4.34-4.40 months (CORAL) for the FAS, and 8.25 (JULIET) vs. 4.04-4.86 (CORAL) for the ITT populations. Tisagenlecleucel was associated with a significantly higher ORR compared to historical treatments among the FAS (adjusted response rate difference [95% CI], both FSW and SMRW: 36% [22%, 0.48%]; p&lt;0.001) and among the ITT populations after SMRW adjustment (11% [0%, 22%]; p=0.043). This analysis supports that improved response and OS are achieved in r/r DLBCL patients treated with tisagenlecleucel when compared to those treated with alternative historical treatments.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5608-5608
Author(s):  
Myrna Candelaria ◽  
Juan Labardini ◽  
Ana Florencia Ramirez ◽  
Alejandro Aviles ◽  
Enrique Estrada-Lobato ◽  
...  

Abstract The actual standard of care of Diffuse Large B-Cell Lymphoma (DLBCL) includes rituximab in combination with chemotherapy, with overall response rates  up to 76 %.  However, this treatment may not be accessible to many patients because of limited economical resources, particularly in  developing countries, where most of the treatment must be paid from the pocket of patients or their families.   In Mexico, since 2011 a Federal program has fully covered   the treatment of patients with DLBCL. At the Instituto Nacional de Cancerología (INCan) in Mexico city 214  patients with ths disease have been treated without cots with the standard of care.   The   mean age at diagnosis was 56.7 +15.9 (22-91). This series of cases was compared with a retrospective analysis of cases with DLBCL seen at the INCan between 2006-2008  None A total of 264 cases were retrospectively analyzed.  No differences were found in demographic and clinical characteristics at the time of diagnosis. However, a clear  positive impact was found in the group that received full treatment thanks to this new social coverage by this new social security program. The follow-up and completion of treatment was 99%.,   In contrast: from 264 in the retrospective group, 209 (79 %)  were treated, but only 29 (10.9 %) were able to receive an optimal treatment, including rituximab.  These differences in treatments had a clearly impact   on the response rate, as shown in the following table:A: Retrospective cases.N (%) B: Cohort.N (%) p Patients. 264 214 -- Treatment:CHOP-R (standard of   care)CHOP0-2 cycles** 2918055** 20410*-- 0.001 Response (all   patients) :CompletePartialStable diseaseProgressiveNot evaluable, lost   of follow up 103  (39)31 (11.7)11 (4.1 )24  (9)95 (36) 124 (58)19 (8.8)0 --29 (13.5)-- 0.001 Relapse rate (%) 93 (35 %) 43 (20%) 0.0001 *7 patients did not received rituximab because of HBV & 3 HIV positive had < 100 absolute CD4 counts.**Patients who abandoned treatment because of reduced economical resources. These results demonstrate the importance of social programs that may accessible   standard treatment options in countries with limited resources. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 39 (S2) ◽  
Author(s):  
L. Pezzullo ◽  
G. Cassiordor0 ◽  
R. Rosamilio ◽  
I. Ferrara ◽  
S. Luponio ◽  
...  

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