Lymphocyte-predominant Hodgkin lymphoma: what is the optimal treatment?

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 406-413 ◽  
Author(s):  
Michelle Fanale
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Radiation Treatment ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
B Cell Lymphomas ◽  
Early Stage Disease ◽  
Stage Disease ◽  
Relapsed Disease

AbstractNodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a unique diagnostic entity, with only ∼ 500 new cases in the United States per year with a similar infrequent incidence worldwide. NLPHL also has distinctive pathobiology and clinical characteristics compared with the more common classical Hodgkin lymphoma (cHL), including CD20 positivity of the pathognomic lymphocytic and histiocytic cells and an overall more indolent course with a higher likelihood of delayed relapses. Given the limited numbers of prospective NLPHL-focused trials, management algorithms historically have typically been centered on retrospective data with guidelines often adopted from cHL and indolent B-cell lymphoma treatment approaches. Key recent publications have delineated that NLPHL has a higher level of pathological overlap with cHL and the aggressive B-cell lymphomas than with indolent B-cell lymphomas. Over the past decade, there has been a series of NLPHL publications that evaluated the role of rituximab in the frontline and relapsed setting, described the relative incidence of transformation to aggressive B-cell lymphomas, weighed the benefit of addition of chemotherapy to radiation treatment for patients with early-stage disease, considered what should be the preferred chemotherapy regimen for advanced-stage disease, and even assessed the potential role of autologous stem cell transplantation for the management of relapsed disease. General themes within the consensus guidelines include the role for radiation treatment as a monotherapy for early-stage disease, the value of large B-cell lymphoma–directed regimens for transformed disease, the utility of rituximab for treatment of relapsed disease, and, in the pediatric setting, the role of surgical management alone for patients with early-stage disease.

Primary Mediastinal B-Cell Lymphoma: The Dilemma of Radiotherapy

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 30-31
Author(s):  
Filipa Saraiva ◽  
Christopher J. Saunders ◽  
Margarida Fevereiro ◽  
Alexandra Monteiro ◽  
Aida B Sousa
Keyword(s):  
B Cell ◽  
Conflicts Of Interest ◽  
Cell Lymphoma ◽  
Early Stage ◽  
Autologous Transplantation ◽  
B Cell Lymphoma ◽  
Molecular Characteristics ◽  
Early Stage Disease ◽  
Free Survival ◽  
Stage Disease

Introduction: According to the WHO classification, primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is a distinct entity, which has a close relationship with nodular sclerosing classical Hodgkin's lymphoma (cHL) in terms of clinical and molecular characteristics and sharing an excellent prognosis. When PMBCL is treated with R-CHOP, the addition of radiotherapy (RT) turns more than 90% of positron emission tomography (PET) positive cases into complete remissions (CR) and increases 5-year overall survival (OS) to 93%. However, the fear of potential late toxicities due to RT, has led an increasing number of centers to favor more intensive regimens like DA-EPOCH-R without RT. This controversy is deepened by the recent comparison between the latter regime with R-CHOP, unfavorable to the DA-EPOCH-R for toxicity reasons. Objective: Evaluate the effectiveness and late toxicities of R-CHOP plus RT in our center and secondarily compare these results with the ones obtained in our cHL patients with bulky and early stage disease. Methods: Retrospective analysis of patients with PMBCL treated between Jan/2007 and Dec/2017 according to clinical characteristics, late toxicities, rate of CR, OS and disease-free survival (DFS) estimated by Kaplan-Meier method. Results: In 32 patients with a median age of 34 years (23-70), 56% were male, 91% had limited stage and 6% had an unfavorable IPI. The rate of CR was 91% (2 patients needed second line therapy to achieve CR) with 2 relapses (at 6 and 11 months, respectively) rescued with autologous transplantation. Three deaths were recorded due to disease progression. With a median follow-up of 86 months, OS and DFS at 10 years were 91% and 93%, respectively. As for late toxicities, besides 2 cases of severe pulmonary fibrosis, there were no other relevant late toxicities registered. These results overlap those obtained in our cHL patients with bulky and early stage disease treated with Stanford V plus RT with an OS and an event free survival of 93% and 84%, respectively. Conclusion: These results support PMBCL's excellent prognosis, parallel to that of cHL. Given the low probability of late toxicities with this regimen our data uphold against the therapeutic strategy of more intensive regimens, that have an increased management complexity and are clinically more toxic. Disclosures No relevant conflicts of interest to declare.

Double Hit Lymphoma: M.D. Anderson Experience

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1776-1776 ◽  
Author(s):  
Yasuhiro Oki ◽  
Mansoor Noorani ◽  
Richard Eric Davis ◽  
Sattva S. Neelapu ◽  
Alma Rodriguez ◽  
...  
Keyword(s):  
B Cell ◽  
Initial Treatment ◽  
Cell Lymphoma ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
Initial Therapy ◽  
Early Stage Disease ◽  
Double Hit Lymphoma ◽  
Stage Disease ◽  
Aggressive B Cell Lymphoma

Abstract Background Double hit lymphoma (DHL), defined as aggressive B-cell Lymphoma with MYC and BCL2 or BCL6 rearrangement, is associated with dismal outcomes. We report our experience at MD Anderson Cancer Center over the last 15 years. This retrospective study is to serve as a historical control, and was approved by Institutional Review Board. Method We reviewed medical records of patients (pts) with aggressive B-cell lymphoma diagnosed between 1998 and 2012. DHL was defined by presence of MYC gene rearrangement with IGH gene or amplification, determined by fluorescence in situ hybridization or cytogenetic analysis, in addition to similar genetic abnormality of BCL2. Result We identified an initial group of 56 cases of DHL, diagnosed between 1998 and 2012. Median age was 62 years (range 18-84). Fifty pts (89%) had stage III/IV disease. Serum LDH was elevated in 37 pts (66%). IPI score was ≥3 in 42 pts (75%). Six pts (11%) had a history of low-grade lymphoma prior to the diagnosis of DHL. C-MYC rearrangement and amplification was observed in 51 (91%) and 5 (9%), respectively. BCL2 rearrangement and amplification was observed in 54 (96%) and 2 (4%), respectively. BCL6 rearrangement was tested in 9 cases, and rearrangement was observed in 2 (4%) cases (triple hit). Initial regimens were CHOP±rituximab (R) (n=24, complete response [CR] rate 50%), hyper-CVAD alternating with methotrexate plus cytarabine ± R (n=20, CR rate 68%), EPOCH+R (n=6, CR rate 83%) and others (n=6, CR rate 50%). A total of 32 (57%) pts achieved CR. Median progression free survival duration was 9 months. Median overall survival (OS) duration was 18 months. The 3-year OS rate was 31% for all pts (Figure A), 50% for pts who achieved CR after initial therapy, and 5% for pts who did not achieve CR. Among pts who did not achieve CR, one pt is alive (4%) beyond 2.5 years after proceeding to allogeneic stem cell transplant (allo-SCT) immediately after achieving partial response to initial therapy. In the 6 pts with early stage disease, 5 (83%) achieved CR after initial induction therapy and did not undergo frontline SCT. One of them had recurrent disease and underwent autologous SCT (auto-SCT) but died of disease. One pt who achieved only PR after initial therapy proceeded to allo-SCT, and remains in remission beyond 2.5 years after SCT. Overall, in pts with early stage disease, 3-year OS rate was 75%. In pts with advanced stage disease (n=50), 27 (54%) pts achieved CR. Median OS duration was 13 months. 3-year OS rate was 25%, Among pts achieving CR after initial treatment (n=27), 9 underwent consolidative auto- (n=8) or allo- (n=1) SCT (median age 53, range 34-68) and 18 did not (median 61, range 18-84). Among pts undergoing frontline SCT, only 1 pt has experienced recurrence so far (4 months after auto-SCT) with a median follow up duration 17 months. However, 2 died without disease progression; one with treatment related lung toxicity (1 month post auto-SCT) and one with other comorbidities (14 months after auto-SCT). In pts achieving CR and undergoing frontline SCT, the 3-year OS rate was 66%. In pts achieving CR and not undergoing frontline SCT, the 3-year OS rate was 43%, but the difference was not statistically significant (Figure B. log-rank test 0.87, hazard ratio 0.82 for frontline SCT [95% CI 0.17-3.94, p=0.8)]. Among 10 pts who had transplant for refractory or relapsed disease (auto n=7, allo n=3), one pt who underwent allo-SCT is alive 5 years after transplant. All others (n=9) died of disease. Conclusion DHL is associated with poor outcome, except rare cases with early stage diseases. Particularly those who do not respond to initial chemotherapy have a uniformly dismal prognosis. In our limited experience, pts with recurrence typically do poorly following SCT, except rare cases of “cure” from allo-SCT. Thus, effective initial induction therapy leading to CR seems to be most effective path to the long-term survival. Consolidative frontline SCT, either autologous or allogeneic, in pts responding to the initial treatment may play a role in disease control. But in our small subset of pts, survival curve of pts undergoing frontline SCT was not significantly different from those who did not, affected by death of other cause. Further identification and analysis of additional pts with DHL is ongoing. Moreover, well designed prospective clinical trial with novel therapeutic approach is desired. Disclosures: Off Label Use: sirolimus - mTOR inhibitor vorinostat - HDAC inhibitor.

Thyroid lymphoma: A single institution’s experience

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 18533-18533
Author(s):  
E. M. Wallace ◽  
D. G. Power ◽  
G. D. Leonard ◽  
D. N. Carney
Keyword(s):  
B Cell ◽  
Hashimoto’S Thyroiditis ◽  
Early Stage ◽  
Combined Modality Therapy ◽  
Significant Proportion ◽  
B Cell Lymphoma ◽  
Hashimoto's Thyroiditis ◽  
Thyroid Lymphoma ◽  
Early Stage Disease ◽  
Hodgkin's Lymphomas

18533 Background: Extranodal lymphomas account for a significant proportion of non-Hodgkin’s lymphomas. Thyroid lymphoma accounts for <2% of these. Lymphoma represents approximately 2% of all malignant thyroid tumours and is almost always of B-cell lineage. The main presenting symptom is a rapidly enlarging goiter and approximately half present with disease limited to the thyroid gland, designated stage IE. Due to its rarity, there is limited information with regard to prognosis or management. Pre-existing chronic autoimmune (Hashimoto’s) thyroiditis is the only known risk factor. Methods: We retrospectively reviewed all cases of thyroid lymphoma found in our institution over a 15 year period (1985–2000). Eleven patients were identified as suitable for analysis. Diagnosis and staging involved a full history and physical examination with relevant serology. Radiological imaging included computerized tomography, plain radiographs and ultrasound. Bone marrow aspirate and biopsy were performed in ten of the patients and patients were then staged according to the Ann Arbour Classification. We specifically analysed patients for their epidemiological features, presentation, treatment and their overall outcome. We compared our data to those from earlier series. Results: Seven of the 11 patients were female with a median age of diagnosis of 56. Most patients complained of neck swelling for a number of months but dysphagia, dyspnoea and hoarseness (80%) were the symptoms that prompted presentation. Hashimoto’s thyroiditis was not found. The commonest histological subtype was diffuse large B cell lymphoma (45% of cases). Seven patients had stage I disease, 3 stage II and 1 stage IV. Nine patients received chemotherapy and radiotherapy was administered in the adjuvant setting to nearly half of the patients. No correlation between presentation and other diagnostic tests or survival was found. Two-year survival for our patients was 100% and no cases died from their disease. Conclusion: Thyroid lymphoma is a rare disease and valuable information on this subject has been provided by single institution studies. Our encouraging survival figures are likely a combination of the predominance of early stage disease and the use of combined modality therapy. The use of monoclonal antibodies may provide a further benefit to these patients. No significant financial relationships to disclose.

scholarly journals Hodgkin lymphoma requires stabilized NIK and constitutive RelB expression for survival

Blood ◽  
2012 ◽  
Vol 120 (18) ◽  
pp. 3756-3763 ◽  
Author(s):  
Stella M. Ranuncolo ◽  
Stefania Pittaluga ◽  
Moses O. Evbuomwan ◽  
Elaine S. Jaffe ◽  
Brian A. Lewis
Keyword(s):  
Cell Viability ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Therapeutic Target ◽  
Cell Lymphoma ◽  
Family Members ◽  
B Cell Lymphoma ◽  
Shrna Expression ◽  
Noncanonical Pathway

Abstract We have analyzed the role of the REL family members in Hodgkin lymphoma (HL). shRNA targeting of each REL member showed that HL was uniquely dependent on relB, in contrast to several other B-cell lymphomas. In addition, relA and c-rel shRNA expression also decreased HL cell viability. In exploring relB activation further, we found stable NF-κB inducing kinase (NIK) protein in several HL cell lines and that NIK shRNA also affected HL cell line viability. More importantly, 49 of 50 HL patient biopsies showed stable NIK protein, indicating that NIK and the noncanonical pathway are very prevalent in HL. Lastly, we have used a NIK inhibitor that reduced HL but not other B-cell lymphoma cell viability. These data show that HL is uniquely dependent on relB and that the noncanonical pathway can be a therapeutic target for HL. Furthermore, these results show that multiple REL family members participate in the maintenance of a HL phenotype.

scholarly journals A Retrospective Analysis of Outcomes of Diffuse Large B Cell Lymphoma (DLBCL) in the Elder Patients in China

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5410-5410
Author(s):  
Peng Liu ◽  
Ying Han ◽  
Jianliang Yang ◽  
Xiaohui He ◽  
Changgong Zhang ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
Disease Stage ◽  
Early Stage Disease ◽  
Large B Cell Lymphoma ◽  
Elder Patients ◽  
Stage Disease ◽  
Ann Arbor ◽  
Advanced Disease Stage

Abstract Background: Elderly patients with diffuse large B-cell lymphoma (DLBCL) have a worse prognosis compared to the younger population, since older age is associated with comorbidity and suboptimal performance status leading to intolerance of chemo-immunotherapy. The outcome of DLBCL in the older patients (> 60 years) was well described in clinical trials with reported 5-year overall survival (OS) of 50-80% (Coiffier et al., N Engl J Med 2002). Since this group is often precluded from clinical trials and population-based studies are limited, optimal treatment strategy for the old patients with DLBCL remains controversial. Here, we describe a Chinese real-world experience of management of elderly DLBCL patients treated at National Cancer Hospital, China. Methods: This is a single-center, retrospective analysis of consecutive DLBCL patients aged ≥60 who planned to receive chemotherapy +/- rituximab. The standard regimens included 3-4 cycles (early stage disease) or 6 cycles (advanced) of R-CHOP like regimens (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) followed by two rituximab doses in fit patients; R-miniCHOP for unfit and R-CE(etoposide)OP for frail elder patients. Patient data including baseline characteristics, histology, clinical parameters, and treatment outcomes were extracted from hospital medical records. The primary endpoint was progression-free survival (PFS); secondary endpoint was OS. Statistical analyses included descriptive statistics and Kaplan Meier estimates. Results: From June 2006 to December 2012, 349 patients aged ≥60 years were included, in which 204 patients were aged <70 years (Table 1). 326 patients received chemotherapy or chemo-immunotherapy with rituximab. Median follow up was 82 months. Five year PFS and OS of the elder patients were 45.8% and 51.9%, respectively. Significant difference was seen between patients < 70 years and those ≥70 years in terms of PFS (51.0% vs 38.6%, p=0.030) and OS (58.3% vs 42.8%, p=0.007) (Figure 1). Patients with early-stage disease (Ann Arbor StageⅠ/Ⅱ) had better 5-year PFS (60.1% vs 23.5%, p<0.001) and OS (65.3% vs 30.9%, p<0.001) than patients with advanced disease stage (Ann Arbor Stage III/IV) (Figure2). In addition, regimen including rituximab significantly improved the survival than chemotherapy alone (5-year PFS: 37.3% vs 64.0%, p<0.001; 5-year OS: 44.5% vs 69.3%, p<0.001), especially in patients ≥70 years, which almost doubled 5-year PFS and OS (5-year PFS: 25.4% vs 50.7%, p<0.001; 5-year OS: 28.8% vs 56.0%, p<0.001) (Figure3). Conclusions: Elder age (≥70 years) and advanced disease stage (Ann Arbor Stage III/IV) are associated with poor PFS and OS in Chinese elder DLBCL patients. The addition of rituximab significantly improves the survival compared to chemotherapy alone, especially in patients aged ≥70 years. These findings underscore the importance of personalized evaluation and treatment in elder patients with DLBCL. Disclosures No relevant conflicts of interest to declare.

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