Nongastric Marginal Zone B-Cell Lymphoma: Analysis of 247 Cases - Clinical Presentation and Treatment Outcomes of Nongastric Marginal Zone B-Cell Lymphoma.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1491-1491
Author(s):  
Sung Yong Oh ◽  
Baek-Yeol Ryoo ◽  
Won Seog Kim ◽  
Yeon Hee Park ◽  
Kihyun Kim ◽  
...  

Abstract Purpose: Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. We report a retrospective analysis of 247 patients with .NG-MZL, presenting their clinical features and therapeutic outcomes. Methods: From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Results: The median age was 49 years (range, 13–89 years). The study involved 129 males (52.2%) and 118 females (47.8%) The most common involving site was orbit and ocular adnexa (48.6%) followed by lymph node and lymphatic organs (17.8%), bowel (9.3%), lung (6.1%), thyroid gland (4.9%), salivary gland (4.5%) in the decreasing order of frequency. Ann Abor stage I/II disease was present in 78%(167 out of 215). BM involvement was less than 10%(19 out of 211). B symptom was observed in only 2%. One and eighty-six patients out of 208 were in low or low-intermediate risk group (89%) according to international prognostic index(IPI). Eighty percents (172/215) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Patients were treated with a variety of therapeutic strategies. Complete and partial remissions were achieved in 139(92%) and 8(5.3%) of the 151 stage I/II patients, respectively, with an overall response rate of 97.3%. Especially, radiation containing treatment achieved 96% CR rate (108 out of 113). In 38 patients with stage III/IV, CR and PR were achieved in 17(44.7%) and 11(28.9%). The estimated 5-year overall survival (OS) and progression free survival (PFS) were 93.8% and 70.1%, respectively. Although anthracyclin contaning regimen could achieve higher CR rate, it did not improve PFS. Stage III/IV, low hemoglobin, high/high-intermediate IPI, poor risk FLIPI and nodal MZL were poor prognostic factors for PFS in multivariate analysis. Conclusion: NG-MZL is an indolent disease. For localized disease radiation achieved excellent local control. Anthracyclin containing chemotherapy cannot improve outcome. Both IPI and FLIPI can be applied to predict the prognosis.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1361-1361
Author(s):  
Sung Yong Oh ◽  
Hyuk-Chan Kwon ◽  
Won Seog Kim ◽  
Yeon Hee Park ◽  
Kihyun Kim ◽  
...  

Abstract Purpose: The International Prognostic Index (IPI) and Follicular Lymphoma Prognostic Index (FLIPI) are used as prognostic indices for NHL and indolent lymphoma. However, marginal zone B-cell Lymphoma (MZL) evidences a distinctive clinical presentation and a natural course; thus, in this study, we attempted to devise an adequate prognostic index for MZL. Patients and method: From 1990 to 2005, 205 patients diagnosed with MZL were retrospectively reviewed. After analysis of the prognostic factors, progression free survival (PFS) and overall survival (OS), we constructed a prognostic index of MZL (MZLPI) via the summation of each factor. We then compared PFS and OS with IPI, FLIPI, and MZLPI. Results: According to our multivariate analysis of PFS and OS of MZL, nodal MZL, ECOG performance ≥ 2 and advanced stage were composed of MZLPI. MZLPI was grouped as follows: score 0 as a low risk group, score 1 as an intermediate risk group, and score ≥2 as a high risk group. The PFS curve, according to MZLPI results, evidenced a more discriminated pattern than IPI and FLIPI, and this was especially true in the intermediate risk group. In OS, MZLPI (P=0.0007) evidenced a more discriminated pattern than IPI (P=NS) or FLIPI (P=0.0044). Conclusion: MZLPI, which is constructed of relatively simple factors, may represent a useful prognostic index for the prediction of PFS and OS in MZL, and may also be used as a substitute for IPI or FLIPI.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4742-4742
Author(s):  
Sung Yong Oh ◽  
Won Seog Kim ◽  
Baek-Yeol Ryoo ◽  
Hye Jin Kang ◽  
Yeon Hee Park ◽  
...  

Abstract Purpose: Intestinal marginal zone B-cell lymphoma of MALT type (I-MZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and optimal treatment modality has not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of I-MZL. Methods: From 1990 to 2005, a total of 27 patients with histologically confirmed I-MZL were analyzed. Results: The median age was 51 (range: 19–83) years. The study involved 16 males (59%) and 11 females (41%). Patients initially presented with abdominal pain (62.9%), diarrhea (22.2%) and various symptoms. The most common involving site was ileo-cecal area (40.7%) followed by small intestine (25.9%), colorectal (18.5%), multiple intestine involvements (14.8%) in the decreasing order of frequency. Musshoff stage IE, IIE1, IIE2, IIIE, and IV were present in 44%, 15%, 11%, 7.4%, and 22%, respectively. B symptom was observed in 11%. Twenty one (78%) and five (19%) patients were in low or low-intermediate risk group according to international prognostic index (IPI). Sixty-three percents (17/27) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Treatment was mainly combined with surgical modality. Complete and partial remissions were achieved in 22 (82%) and 1(4%). After the median follow-up duration of 56.1 months, Median progression free survival (PFS) were 8.8 (95% CI: 3.8–13.8) years. The estimated 5-year overall survival (OS) and PFS were 86% and 54%, respectively. Stage ≥ IIE2 was poor prognostic factor of PFS and OS. Conclusion: I-MZL trends to be an indolent disease - prolonged survival with frequent relapse like other site MZL of MALT type. They present commonly early stage, low risk state and respond well local and systemic treatment. I-MZL has favorable prognosis.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1563-1563
Author(s):  
Laura S. Jacobus ◽  
Julianne Lunde ◽  
Matthew J Maurer ◽  
Ahmet Dogan ◽  
Sergei I Syrbu ◽  
...  

Abstract Abstract 1563 Intro: Optimal treatment for non-follicular low grade B-cell lymphoma (NFLGL) is not well defined. Clinical trial design for these subtypes would be enhanced by further understanding of early presentation and management. A large prospective observational study was used to identify presenting clinical features, describe patterns of care and compare the prognostic utility of International Prognostic Index (IPI) and the Follicular Lymphoma International Prognostic Index (FLIPI). Methods: The University of Iowa/Mayo Clinic SPORE Molecular Epidemiology Resource (MER) is a prospective, longitudinal observational study designed to collect information on patterns of care and outcomes for patients with newly diagnosed lymphoma. Patients seen at the Mayo Clinic, Rochester MN and the University of Iowa within 9 months of their initial diagnosis of lymphoma are offered enrollment. Baseline patient reported data, clinical data and initial management are collected using a standard protocol and a central pathology review is performed. After enrollment, patients are actively followed for events (disease progression, retreatment, and death) and disease related comorbidities. This analysis includes 198 patients diagnosed with extranodal marginal zone (EMZL), 22 with nodal marginal zone (NMZL), 47 with splenic marginal zone (SMZL), 48 with lymphoplasmacytic lymphoma (LPL), as well as 67 patients with unclassifiable low-grade B-cell lymphoma (B-NOS). Results: 382 newly diagnosed NFLGBL patients were enrolled in the MER from 2002–2009. The median age at enrollment was 63 years (range 22–95). 52% were male. The most common types of initial therapy after diagnosis were observation (41 %), alkylator- based chemotherapy +/− rituximab (R) (17%), radiation therapy alone (15%) and R monotherapy (13%). At median follow-up of 60 months (range 1–121), 51 patients were deceased and 155 had an event. The clinical characteristics that comprise the IPI and FLIPI were similar across the subtypes. Approximately half of the cases presented at low risk (55% IPI; 48% FLIPI). A majority had a normal LDH (81%), limited nodal involvement (91% had ≤ 4 groups), normal hemoglobin (71% ≥12 g/dL), good performance status (95% 0–1), and advanced stage (57% stages III-IV). There were notable differences among the NFLGBL subtypes. Patients with SMZL and LPL presented with high risk FLIPI of 3–5 (49% and 46% respectively) and high to high-intermediate IPI of 3–5 (21% and 13%). Abnormal LDH and anemia (< 12 g/dL) were more frequent in patients with SMZL (40%; 56%) and LPL (25%; 62%). EMZL presented with lower stages (70% stage I-II) and NMZL presented with increased nodal involvement (38% >4 sites). SMZL had the highest rates of surgical resection (21%) and EMZL was more frequently treated with radiation therapy (25%). LPL (30%) and NMZL (32%) more commonly received alkylator based chemotherapy +/− R. While both IPI and FLIPI differentiated outcome in these patients, FLIPI had higher c-statistics than IPI for both EFS (0.583 vs 0.568) and OS (0.685 vs 0.661), respectively. Conclusions: This large prospective collection of data on NFLGBL patients presenting to academic medical centers provides a modern picture of presentation and management patterns useful to investigators designing clinical trials. FLIPI may have more prognostic utility than IPI in this group of patients. Disclosures: Ansell: Seattle Genetics, Inc.: Research Funding.


2021 ◽  
Vol 104 (2) ◽  
pp. 003685042110225
Author(s):  
Yun Lian ◽  
Jiayu Huang ◽  
Huihui Zhao

This retrospective study was designed to describe the clinical characteristics and prognosis of human immunodeficiency virus (HIV)-infected diffuse large B-cell lymphoma (DLBCL) patients. We retrospectively enrolled 31 patients newly diagnosed with HIV-infected DLBCL from 2009 to 2019 in our institution. The median age of patients was 47 years, and most patients were male ( n = 27, 87.1%). Baseline mean CD4+ count was 150.72 ± 146.57/μl. Eighteen (58.1%) patients had B symptoms. Categorized by international prognostic index (IPI) score, 7 cases (22.6%) were in low-risk group (IPI 0-1) and 24 cases (77.4%) were in medium-high risk group (IPI 2-5). Twenty-five (80.6%) patients received highly active antiretroviral therapy (HAART) and 16 (51.6%) underwent standard chemotherapy. The mortality rate was 58.1% (18/31). Univariate survival analysis revealed that HCV infection ( p = 0.032), standard chemotherapy treatments ( p = 0.038) were associated with overall survival (OS). Our results showed that HIV-infected DLBCL patients had high-risk stratification and high mortality. HCV-coinfection might be associated with poor OS. Early diagnosis and standardized treatments might be beneficial for promoting the survival of HIV-infected DLBCL patients.


2019 ◽  
Vol 72 (1-2) ◽  
pp. 25-29
Author(s):  
Maja Popovic ◽  
Gorana Matovina-Brko ◽  
Dragana Petrovic ◽  
Bojana Vranjkovic ◽  
Jelena Radic ◽  
...  

Introduction. The research aimed to evaluate the impact of age-adjusted international prognostic index and time to the first relapse on overall survival and progression-free survival from the beginning of the second line of treatment in patients with relapsed/ refractory diffuse large B-cell lymphoma. Material and Methods. The research included 36 patients with relapsed/refractory diffuse large B-cell lymphoma treated at the Oncology Institute of Vojvodina, Serbia, from January 2013 to December 2015. Patients were stratified according to age-adjusted international prognostic index score at the time of relapse into patients with low risk (score 0 - 1) and patients with high risk (score 2 - 3), as well as according to the time of the first relapse: early relapse (? 12 months) and late relapse (> 12 months). Results. In the group of patients with a score of 0 - 1, the median overall survival was 44 months compared with 6 months in patients with score of 2 - 3, hazard ratio 0,4 (confidence interval 0,16 - 0,99), p = 0,03. In patients with early relapse, the median overall survival was 7 months compared with 25 months in patients with late relapse, hazard ratio 0,55 (confidence interval 0,25 - 1,19), p = 0,12. In patients with early relapse, median progression-free survival was 0 months compared with 10 months in patients with late relapse, hazard ratio 0,34 (confidence interval 0,12 - 1,00), p = 0,0017. Conclusion. The impact of age-adjusted international prognostic index score significantly affects overall survival in patients with relapsed diffuse large B-cell lymphoma. The time to the first relapse impacts progression-free survival calculated from the time of the second-line treatment initiation.


2021 ◽  
Author(s):  
Zhengzi Qian ◽  
Leiyuan Chen ◽  
Xinyuan Wang ◽  
Yutian Kan ◽  
Yafei Wang ◽  
...  

Abstract Metastasis-associated lung adenocarcinoma transcript 1 (MALAT1) has been involved in the pathogenesis and progression of several cancers. However, the exact effect of MALAT1 in primary gastrointestinal diffuse large B-cell lymphoma (PGI-DLBCL) has not been elucidated. This study aimed to explore the prognostic value of MALAT1 in PGI-DLBCL patients. Quantitative real-time Polymerase Chain Reaction (qRT-PCR) was performed to detect the expression of MALAT1 in 90 patients with PGI-DLBCL. MALAT1 was remarkably up-regulated in PGI-DLBCL tissues as compared to that of paired adjacent non-tumor tissues (P<0.001), and the area under the ROC curve (AUC) was 0.838. MALAT1 expression was further increased in the non-germinal center B-cell-like (non-GCB) group, advanced stage (stages IIE-IV) group and International Prognostic Index (IPI) score (3-5) group (P=0.01, P<0.001and P<0.001, respectively). Furthermore, Kaplan-Meier analysis showed that elevated MALAT1 expression was correlated with inferior Overall survival (OS) and progression free survival (PFS) in PGI-DLBCL patients (P<0.001and P<0.001, respectively), and multivariate analysis suggested that up-regulation of MALAT1 and high IPI score (3-5) were two unfavorable prognostic factors of PGI-DLBCL. In conclusion, our results demonstrates that MALAT1 might serve as a novel prognostic biomarker and an ideal therapeutic target for PGI-DLBCL patients in the future.


2008 ◽  
Vol 26 (19) ◽  
pp. 3189-3195 ◽  
Author(s):  
Kazuyuki Shimada ◽  
Kosei Matsue ◽  
Kazuhito Yamamoto ◽  
Takuhei Murase ◽  
Naoaki Ichikawa ◽  
...  

Purpose To evaluate the safety and efficacy of rituximab-containing chemotherapies for intravascular large B-cell lymphoma (IVLBCL). Patients and Methods We retrospectively analyzed 106 patients (59 men, 47 women) with IVLBCL who received chemotherapy either with rituximab (R-chemotherapy, n = 49) or without rituximab (chemotherapy, n = 57) between 1994 and 2007 in Japan. The median patient age was 67 years (range, 34 to 84 years). The International Prognostic Index was high-intermediate/high in 97% of patients. Results The complete response rate was higher for patients in the R-chemotherapy group (82%) than for those in the chemotherapy group (51%; P = .001). The median duration of follow-up for surviving patients was 18 months (range, 1 to 95 months). Progression-free survival (PFS) and overall survival (OS) rates at 2 years after diagnosis were significantly higher for patients in the R-chemotherapy group (PFS, 56%; OS, 66%) than for patients in the chemotherapy group (PFS, 27% with P = .001; OS, 46% with P = 0.01). Multivariate analysis revealed that the use of rituximab was favorably associated with PFS (hazard ratio [HR], 0.45; 95% CI, 0.25 to 0.80; P = .006) and OS (HR, 0.42; 95% CI, 0.21 to 0.85; P = .016). Treatment-related death was observed in three patients (6%) who received R-chemotherapy and in five patients (9%) who received chemotherapy. Conclusion Our data suggest improved clinical outcomes for patients with IVLBCL in the rituximab era. Future prospective studies of rituximab-containing chemotherapies are warranted.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3447-3447
Author(s):  
Daisuke Ennishi ◽  
Masahiro Yokoyama ◽  
Kengo Takeuchi ◽  
Hiroaki Asai ◽  
Yuko Mishima ◽  
...  

Abstract Background: Several biomarkers indicating poor prognosis have been reassessed in patients with diffuse large B-cell lymphoma (DLBCL) treated by rituximab combination chemotherapy. However, no studies have investigated the outcome by combining these biomarkers for rituximab treatment. In addition, no large studies have reassessed the outcome of patients with CD5-positive DLBCL treated by rituximab. To determine the most influential biomarkers, we reassessed and investigated the predictive value of three biomarkers, namely Bcl-2 expression, GC phenotype and CD5 expression, in DLBCL patients treated with rituximab in combination with CHOP as an initial therapy. Methods: A total of 121 patients with CD20-positive DLBCL receiving RCHOP at our institute between April 2002 and October 2005 were enrolled in the study. To classify the samples into immunohistochemically defined GC or non-GC phenotypes, we used a previously described algorithm using expression of CD10, Bcl-6, and MUM1 (Hans et al. Blood, 2004). For examination of the CD5 expression, we defined the cases as CD5-positive if CD5 expression was detected by immunohistochemical and flow cytometric analyses. We also assessed the outcome of patients according to International Prognostic Index (IPI). Results: Expression of Bcl-2 was detected in 79 of the 121 (65%) patients, while CD5 expression was positive in 11 patients (9%). Overall, 48 of the 121 (40%) patients expressed a non-GC phenotype. CD5-positive patients displayed a significantly poorer progression-free survival (PFS) and overall survival (OS) than CD5-negative patients (2-year PFS, 18% vs. 73%, P<0.001; OS, 45% vs. 91%, P=0.001). However, there were no significant differences in PFS and OS according to Bcl-2 expression (PFS, 76% vs. 91%, P=0.08; OS, 77% vs. 97%, P=0.06) or GC phenotype (PFS, 70% vs. 90%, P=0.08; OS, 77% vs. 91%, P=0.12). In contrast, the differences between high or high-intermediate and low or low-intermediate of IPI for PFS and OS were significant (2-year PFS, 52% vs. 91%, P=0.001; OS, 64% vs. 92%, P=0.01). Multivariate analysis revealed that CD5 expression and the IPI were significant prognostic factors for PFS and OS (RR: PFS, 13.57 and 9.65, respectively; OS, 18.15 and 10.72, respectively) (Table 1). Conclusion: These results demonstrated that CD5 expression was the most influential prognostic factor among the biomarkers examined and associated with a very poor outcome, even after rituximab treatment. To confirm this conclusion, further large studies of CD5-positive DLBCL patients are required. Cox’s hazard regression analysis of PFS and OS Variable RR 95% CI P RR indicates relative risk; CI, confidence interval; GC phenotype, non-GC type worse; and IPI, higher IPI worse PFS CD5 13.57 4.260–42.947 <0.001 BCL2 2.00 0.518–7.761 0.314 GC phenotype 2.59 0.851–7.857 0.094 IPI 0 to2 or 3 to 5 9.65 2.911–31.987 <0.001 OS CD5 18.15 3.023–109.029 0.002 BCL2 1.29 0.240–6.966 0.764 GC phenotype 1.43 0.367–5.573 0.606 IPI 0 to2 or 3 to 5 10.72 1.945–59.085 0.006


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 7557-7557
Author(s):  
J. Walewski ◽  
G. Mead ◽  
A. Jack ◽  
S. Barrans ◽  
J. Radford ◽  
...  

7557 Background: Previous studies suggest that CODOX-M/IVAC is effective therapy for BL (Ann Onc 2002 13:1264–74), however the diagnosis of BL in this and other studies was not based on modern immunochemistry and cytogenetics and is unreliable. To re-evaluate this question we prospectively studied a population of patients (pts) with aggressive B-cell lymphoma (100% Ki-67+) uniformly treated with dr CODOX-M/IVAC. Methods: Pts ≤65 years with B-cell lymphomas showing 100% Ki-67, considered fit for chemotherapy, received either dr CODOX-M x 3 or dr CODOX-M/IVAC x 4 according to a modified international prognostic index (IPI). Chemotherapy was modified by methotrexate dose reduction to 3g/m2. Pts >65 years had further dose reductions; unfit pts were studied pathologically only. Tumours were characterised using both an extended panel of antibodies and interphase FISH on paraffin sections for the presence of the C-MYC and BCL-2 rearrangements. Results: Of 126 pts reviewed centrally, 5 were ineligible; 53 were diagnosed as BL, each based on the combination of the presence of re-arrangement of C-MYC as a sole abnormality, germinal centre phenotype and p53 abnormality. The final 68 cases were highly heterogenous with respect to tumour phenotype and cytogenetics and were diagnosed as diffuse large B-cell lymphoma (DLBCL). Median age (all pts) was 44 years (range 17–83), with 23 aged >65. Compared with the DLBCL pts, BL pts were significantly younger (mean 38yrs vs 53 yrs, p < 0.001), had more marrow involvement (45% vs 24%, p = 0.02) and male predominance (83% vs 65%, p = 0.03). Of 104 pts entered into the clincal study, 32 pts (10 BL, 22 DLBCL, IPI 0,1) received dr CODOX-M x 3 and 72 (39 BL, 33 DLBCL, IPI >1) received dr CODOX-M/IVAC x 4. With median follow-up of 15 months (range 1 to 37), 1 year progression-free survival was 58%, 95% CI 48%-68% (54% BL vs 62% DLBCL) and 1 year survival 61% 95% CI 51%-71% (55% BL vs 66% DLBC). Conclusions: The study shows Ki67 is not an accurate approach to the diagnosis of BL and the use of immunocytochemistry and FISH is essential. BL and DLBCL as defined differ markedly clinically. Preliminary data suggest that dr CODOX-M/IVAC has similar activity in both histologies. No significant financial relationships to disclose.


Blood ◽  
2006 ◽  
Vol 109 (5) ◽  
pp. 1857-1861 ◽  
Author(s):  
Laurie H. Sehn ◽  
Brian Berry ◽  
Mukesh Chhanabhai ◽  
Catherine Fitzgerald ◽  
Karamjit Gill ◽  
...  

Abstract Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous entity, with patients exhibiting a wide range of outcomes. The addition of rituximab to CHOP chemotherapy (R-CHOP)has led to a marked improvement in survival and has called into question the significance of previously recognized prognostic markers. Since randomized controlled trials of R-CHOP in DLBCL have included select subgroups of patients, the utility of the International Prognostic Index (IPI) has not been reassessed. We performed a retrospective analysis of patients with DLBCL treated with R-CHOP in the province of British Columbia to assess the value of the IPI in the era of immunochemotherapy. The IPI remains predictive, but it identifies only 2 risk groups. Redistribution of the IPI factors into a revised IPI (R-IPI) provides a more clinically useful prediction of outcome. The R-IPI identifies 3 distinct prognostic groups with a very good (4-year progression-free survival [PFS] 94%, overall survival [OS] 94%), good (4-year PFS 80%, OS 79%), and poor (4-year PFS 53%, OS 55%) outcome, respectively (P < .001). The IPI (or R-IPI) no longer identifies a risk group with less than a 50% chance of survival. In the era of R-CHOP treatment, the R-IPI is a clinically useful prognostic index that may help guide treatment planning and interpretation of clinical trials.


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