Clinical characteristics and outcomes of ocular adnexal mantle cell lymphoma

Orbit ◽  
2022 ◽  
pp. 1-8
Clara M. Castillejo Becerra ◽  
Lauren A. Dalvin ◽  
Dragan Jevremovic ◽  
David O. Hodge ◽  
Andrea A. Tooley
2020 ◽  
Vol 20 ◽  
pp. S257-S258
Yucai Wang ◽  
Aung Tun ◽  
Alessia Castellino ◽  
David Inwards ◽  
Thomas Witzig ◽  

2007 ◽  
Vol 24 (4) ◽  
pp. 413-418 ◽  
M. Todorovic ◽  
M. Pavlovic ◽  
B. Balint ◽  
N. Kraguljac ◽  
B. Mihaljevic ◽  

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1581-1581
Michael A. Thompson ◽  
Tapan M. Kadia ◽  
B. Nebiyou Bekele ◽  
Michael Wang ◽  
Jorge E. Romaguera

Abstract Background Mantle cell lymphoma (MCL) is an aggressive lymphoma with poor prognosis. Prognostic and predictive molecular markers may aid in identification of patients that may benefit from clinical trials of novel therapies. Cyclin D1 is a highly specific marker of MCL. Alternative splicing in the cyclin D1 gene (CCND1) is modulated by a frequent A/G polymorphism located within the splice donor region of exon 4. CCND1 genotype is associated with the age of onset of HNPCC and with shortened event free survival and greater risk of local relapse in resectable non-small cell lung cancer. Methods Samples were collected from 90 MCL patients treated with R-HyperCVAD from 1999 to 2002. The DNA was produced from peripheral blood lymphocytes and bone marrow using the Genomic extraction Kit (Promega, Madison, WI). The CCND1 exon 4 (codon 242) polymorphism was evaluated using pyrosequencing in the M. D. Anderson Cancer Center core facility as previously described (Kong et al. Cancer Res60:249–52, 2000). Genotype groups were correlated with clinical characteristics under an IRB approved protocol. Results The CCND1 genotype frequency for 90 MCL patients was G/G 20%, G/A 54%, A/A 26%. Clinical characteristics were not statistically significantly different between genotype groups. 89 patients were evaluable for response. The ORR at 6 cycles was 100% for all subjects. The CR+CRu rates were G/G 95%, G/A 85%, A/A 96% (p=0.048). The PR rate was higher for patients with genotype G/A (15%) compared to G/G (6%) and A/A (4%). There were no statistically significant differences with respect to failure free survival (FFS) between groups (p=0.361), although there was a trend for a higher FFS in the A/A genotype group. In addition, overall survival (OS) was higher for A/A genotype patients at 61 vs. 49 months for the other genotypes (p=0.01). Discussion In a previous study of 42 MCL patients the CCND1 genotype frequency was: G/G 31%, G/A 55%, and A/A 14% (Howe and Lynas. Haematologica86:563–9, 2001). They showed that CCND1 polymorphisms did not affect prognosis. However, the chemotherapy regimens were not defined in that study. In our study, a larger number of patients were treated homogeneously with the chemotherapy regimen of R-HyperCVAD. Response rates were high and differed slightly by CCND1 polymorphism. The OS was increased for the A/A genotype with a trend for higher FFS. The discrepancy with prior studies may be related to subject genotype frequencies, treatment regimen, and other factors. The CCND1 polymorphisms may have a different predictive yield with other treatment regimens - eg, bortezomib or mTOR inhibitors. Conclusions Response rates were high in all CCND1 genotype groups, but CR rates were lower in the G/A genotype patients. The OS was increased for the A/A genotype with a trend for higher FFS. Future studies should evaluate the role of CCND1 polymorphisms with other active agents against MCL.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5067-5067
Shuhua Yi ◽  
Zengjun Li ◽  
Gang An ◽  
Chengwen Li ◽  
Dehui Zou ◽  

Abstract Introduction The clinical characteristics and cytogenetic aberrations of mantle cell lymphoma (MCL) is well described in the West, but not in China, and also not mentioned in patients with bone marrow involvement (BMI). The aim of this study was to characterize the clinical and cytogenetic feature of Chinese patients with MCL. Methods During the period July 2003 through November 2012, 50 MCL patients with BMI were diagnosed at our Hospital. Cytogenetic aberrations were detected by FISH on bone marrow cells, using a panel probes including Rb1, TP53, ATM and c-MYC. Results The median age of the 50 patients was 55.5 years at diagnosis, with 38 male patients (76%). Eighteen patients had B symptom, 36 patients with splenomegaly, while 4 patients had hepatomegaly. Twenty-four of the forty-five patients had elevated serum LDH. According to MIPI system, 13 patients (27.7%) were classified into medium risk group, while 34 patients (72.3%) in the high risk prognosis group. In aspect of the cytogenetic aberrations, eleven of forty-two patients (26.2%) had Rb-1 deletion, 7/39 patients (17.9%) with ATM deletion, 16/42 patients (38.1%) with TP53 deletion, while 15/37 patients (40.5%) had c-MYC abnormality, including amplification and translocation. With a median follow-up of 21.5 months, the median estimated progression-free survival (PFS) was 15 months (95% CI 8.6-21.4), and the median estimated overall survival (OS) was 27 months (95% CI 17.5-36.5). Using the Kaplan-Meier method, the MIPI high risk group, deletion of Rb-1, ATM, TP53 and c-MYC abnormality were the adverse prognostic factors for PFS, while deletion of Rb-1, ATM, TP53 and c-MYC abnormality predicted the worse OS in the univariate analysis. All other clinical characteristics did not significantly influence the PFS and OS(p>0.05). TP53 deletion and c-MYC abnormality were the independent prognostic factors for both of PFS and OS in the multivariate analysis. Conclusions The outcome of MCL with BMI was poor. TP53 deletion and c-MYC abnormality were common in MCL with BMI and represented the worst factors for survival. Disclosures: No relevant conflicts of interest to declare.

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4481-4481
Yang Han ◽  
Xin Zhang ◽  
Yu Nie ◽  
Yujie Jiang ◽  
Xiaohui Sui ◽  

Abstract Introduction: Mantle cell lymphoma (MCL) is a sub-type of B-cell non-Hodgkin Lymphomas (NHL) that characterized by a heterogenous clinical course and poor prognosis. The transcription factor paired-box 5 (PAX5) is associated with B cell normal differentiation and development. Herein, we aim to explore both the prognostic factors and the role of PAX5 expression in MCL patients from single-center, which can provide theoretical guidance for clinical practice. Methods: The data of 80 MCL patients admitted to Shandong Provincial Hospital from October 2006 to April 2020 were collected to be analyzed. Kaplan-Meier method and univariate and multivariate cox analysis was used to analyze the correlation between overall survival and prognostic factors. Chi-square test, Pearson and Fisher correlation analysis were performed for statistical analysis of clinical features and experimental indicators. *p value<0.05 indicated that the difference was statistically significant. Immunohistochemistry (IHC) was used to label the protein expression. Gene expression profiles were applied to analyze the discrepancy of PAX5 mRNA expression in some lymphoma types. Results: Clinical characteristics of all MCL patients were analyzed. PAX5 expression was identified by IHC in this study: the positive expression rate of PAX5 in all MCL patients was 60% (Figure 1A). The mRNA expression level of PAX5 was obviously elevated in MCL specimens than in normal group compared with other groups (p= 0.034) (Figure 1B). Besides, CD5, CD19, CD22, CD38, CD79α, CD79β and SOX11 were shown co-expressed with PAX5 by string database analysis (Figure 1C). PAX5-related genes were found mainly enriched in lymphocyte activation, B cell proliferation and NOTCH1 signaling pathway (Figure 1D). As is shown in Figure 1E-I, MIPI score (≥6), median to high risk group, high β2-MG level (≥2.65 mg/L), ECOG score (≥2), and splenomegaly were associated with adverse survival (p= 0.006, 0.030, 0.001, 0.019 and 0.001 respectively). The positive expression of PAX5 indicated a shorter overall survival in MCL patients (p= 0.024, Figure 1G). Positive PAX5 expression was associated with international prognostic index (MIPI) score (p= 0.038), high risk stratification (p= 0.006), WBC count (p= 0.024), and increased β2-microglobulin level (p= 0.008). MCL patients with PAX5-positive expression, high level of β2-MG level (≥2.65 mg/L), splenomegaly correlated with a poorer OS (p=0.002, and 0.004 respectively, Figure 1K, L). In patients with PAX5-negative expression, splenomegaly also indicated poor prognosis (p= 0.030, Figure 1M). Furthermore, among patients with high MIPI scores, PAX5-positive MCL patients had a shorter overall survival than PAX5-negative patients (p= 0.016, Figure N, O). Multivariate analysis showed that positive PAX was an independent prognostic factor for poor survival of MCL (p= 0.035). Conclusions: The positive expression of PAX5 in immunohistochemistry may be a factor contributing to the poor prognosis of MCL patients, which is correlated with clinical characteristics and laboratory indicators to a certain extent. Our results the role of PAX5 positivity in MCL and provide clinical guidance for clinical prognostic risk assessment and treatment strategy selection. Keywords: Mantle cell lymphoma; Paired-box 5; Prognosis; Immunohistochemistry Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3326-3326
Nonglak Kanitsap ◽  
Naree Wannissorn ◽  
Arnuparp Lekhakula ◽  
Jakrawadee Julamanee ◽  
Archrob Khuhapinant ◽  

Abstract Introduction: Mantle cell lymphoma (MCL) is a relatively rare and aggressive mature B-cell non-Hodgkin lymphoma (NHL) entity, encounting for 3-10% of newly diagnosed patients. The purpose of the study is to analyse the incidence, clinical features and outcomes of Thai patients with MCL and compare data with those of Western countries. Patients and methods: From the nationwide multicenter registry of 4,056 newly diagnosed NHL patients in Thailand between 2007-2014, patients with MCL were identified according to the criteria of diagnosis by WHO classification of lymphoid disorders, 2008. The incidence, baseline clinical characteristics and outcomes were then analysed. Results: Ninety-nine patients (2.4%) were found to have MCL. The median age was 59.8 years (range 33-86). Seventy-nine percent of patients were men. The primary sites of disease were lymph nodes (60%), bone marrow (19%), GI tract (9%), Waldeyer's ring (4%), sinonasal area (2%) and miscellaneous (6%). (Figure 1). Eighty-three percent of the patients had stage III/IV. Eighty-one patients (82%) received chemotherapy (60.5% with CHOP or CHOP-like chemotherapy, 30.9% with HyperCVAD, and 26% with rituximab-based). Other received palliative chemotherapy or did not receive any. At a median follow-up time of 42 months, the median survival was 30 months (range, 1-94 months). By using Cox analysis, only Ann Arbor stage had impact on overall survival. Conclusion: MCL is less frequent in Thailand compare to the Western countries. The long-term survival is also much inferior reflecting the limited access to rituximab as well as the high intensity therapy in treating MCL in Thailand. Table 1. Comparison of MCL patients according to treatment modalities Clinical characteristics (Total 81 cases) CHOP-like (n=49 ) High intensity CMT (n = 25 ) CVP or palliative CMT (n = 7 ) p-value No R 33 19 4 - Age > 60 19 (67%) 6 (24%) 5 (71%) - Stage III-IV 42 (85.7%) 21 (84%) 7 (100%) - Response Overall response Complete response 26 (53.1%) 19 (38.8%) 17 (68%) 11 (44%) 6 (85.7%) 3 (42.8%) - Survival Mean OS (months) (Min-Max) 3yr-OS 5yr-OS 26.8 (0.1-83) 52.2% 52.2% 38.8 (0.8-93.9) 51% 38.1% 34.3 (10.7-70.5) 51% 30.2% 0.44 0.44 Figure 1. Biopsy sites Figure 1. Biopsy sites Figure 2. The Kaplan-Meier plots of OS according to treatment groups Figure 2. The Kaplan-Meier plots of OS according to treatment groups Figure 3. The Kaplan-Meier plots of OS according to tretment with rituximab Figure 3. The Kaplan-Meier plots of OS according to tretment with rituximab Disclosures Khuhapinant: Roche: Honoraria.

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