Outcome of Observation As Initial Strategy in Patients with Mantle Cell Lymphoma

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2699-2699 ◽  
Author(s):  
Pau Abrisqueta ◽  
Graham W Slack ◽  
David W Scott ◽  
Randy D. Gascoyne ◽  
Joseph M. Connors ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Early Treatment ◽  
Peripheral Blood ◽  
Research Funding ◽  
Treatment Initiation ◽  
Clinical Presentation ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Ki 67 ◽  
Mantle Cell

Abstract Background Patients with mantle cell lymphoma (MCL) follow a heterogeneous clinical course ranging from very indolent to very aggressive. While patients with MCL generally require treatment initiation shortly after diagnosis, it is unclear whether deferring treatment in patients with "indolent" MCL affects their overall outcome. Because it is difficult to identify such patients at the time of diagnosis, their course can only be retrospectively described as indolent after a prolonged period of observation. The aim of this study was to describe the subgroup of patients with MCL who underwent observation as their initial management, including their clinical and biological characteristics and outcomes. Methods Patients diagnosed with MCL from 1998-2015 who were initially observed for ≥3 months from the date of definitive diagnosis were identified in the BCCA Lymphoid Cancer and Pharmacy Databases. Pathology was centrally reviewed at the time of diagnosis, and only cases positive for CCND1 by immunohistochemistry and/or t(11;14) by FISH were included. During the study period, there were no predefined criteria guiding observation or active treatment. Eventual treatment indications included high tumor burden, disease associated symptoms or peripheral blood cytopenias. Clinical-biological features at diagnosis, treatment and outcomes, were analyzed. Results A total of 725 patients with MCL were initially identified, but 286 were excluded: missing data (n=179), treatment refusal (n=7), no treatment due to frailty (n=16), or absence of CCDN1 or t(11;14) confirmation (n=84). 365 (83%) patients received early treatment (ET) and 74 (17%) were observed >3 months (OBS), as shown in Table 1. In the OBS group, 52 (71%) patients had measurable lymph nodes at presentation, 16 (22%) a non-nodal presentation (defined as peripheral blood, bone marrow, and/or spleen only), and 5 (7%) only had gastro-intestinal involvement. Patients in the OBS group were older, with favorable presenting features including good performance status, less frequent B symptoms or increased LDH, and lower Ki67 (<30% vs ≥ 30%) than the ET group. However, MIPI scores were similar between both groups. The majority of patients received rituximab-containing chemotherapy (most commonly R-CHOP or R-bendamustine) at the time of initial treatment in both the ET group (70%) and the OBS group (72%). In the OBS cohort, with a median follow-up of 47 months (range 3.4 - 158 months) in living patients, the median time to treatment (TtT) was 35.5 months (range 5 - 79 months). 10 patients (14%) were observed for > 5 years without requiring treatment. Factors associated with longer TtT included clinical presentation (non-nodal vs nodal, median not reached vs 29 months; P=.005) and Ki-67 (<30% vs ≥ 30%, median 59 vs 20 months, P=.033). Median OS was significantly longer in the OBS group than in the ET group (66 vs 50 months, respectively, P=.024) reflecting the more favorable disease characteristics of the OBS group. Clinical presentation (ie, non-nodal vs nodal) was the only factor associated with OS (median 123 vs 47 months, P=.003) in the OBS group. Finally, the median OS from date of treatment initiation for patients eventually requiring therapy in the OBS group was 34.4 months. With a median age at treatment initiation of 71 yrs (range 40 - 91 yrs) in the OBS group, OS was not significantly different in comparison with the ET group when the analysis was adjusted by age at treatment. Conclusions A subgroup of patients with MCL may be safely observed at diagnosis of the disease without negatively impacting their outcomes, including not only those patients with non-nodal presentation but also asymptomatic patients with low burden nodal presentation, particularly those with a low proliferative rate. Table 1. Patients characteristics by treatment group Observation (n=74) Early treatment (N=365) p-value Median age, years (range) 68 (39 - 90) 66 (22 - 94) 0.05 Male sex 47/74 (64%) 262/365 (72%) 0.16 Performance status >1 7/71 (10%) 97/337 (29%) <.001 B symptoms 1/73 (1%) 116/353 (33%) <.001 Elevated LDH 5/66 (8%) 110/310 (36%) <.001 Ann Arbor Stage I/II 7/73 (10%) 40/357 (11%) 0.80 Ki-67 ≥30% 6/24 (25%) 89/151 (59%) 0.002 Blastoid morphology 0/74 (0) 44/365 (12%) <.001 Nodular pattern 30/58 (52%) 139/304 (46%) 0.40 MIPI 0.73 -   Low risk 20/64 (31%) 83/288 (29%) -   Intermediate risk 19/64 (30%) 77/288 (27%) -   High risk 25/64 (39%) 128/288 (44%) Disclosures Scott: Celgene: Consultancy, Honoraria; NanoString: Patents & Royalties: Inventor on a patent that NanoString has licensed. Connors:Roche: Research Funding; Seattle Genetics: Research Funding. Savage:Seattle Genetics: Honoraria, Speakers Bureau; BMS: Honoraria; Infinity: Honoraria; Roche: Other: Institutional research funding. Villa:Roche: Research Funding.

Updated Efficacy and Safety, and Exploratory Ki-67 Results For The MCL-001 Study Of Lenalidomide In Mantle Cell Lymphoma Patients Who Relapsed Or Were Refractory To Bortezomib

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3057-3057 ◽  
Author(s):  
Andre Goy ◽  
Michael E. Williams ◽  
Sevgi Kalayoglu Besisik ◽  
Johannes Drach ◽  
Radhakrishnan Ramchandren ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Research Funding ◽  
Cell Lymphoma ◽  
High Dose ◽  
Efficacy And Safety ◽  
High Dose Therapy ◽  
Ki 67 ◽  
Mantle Cell ◽  
Heavily Pretreated

Abstract Introduction Patients with mantle cell lymphoma (MCL) typically respond to initial therapy and almost inevitably relapse with frequent chemoresistance over time and poor outcome. Multiple phase II studies have established the efficacy and safety of lenalidomide, an immunomodulatory agent with tumoricidal and antiproliferative properties, in relapsed/refractory MCL. The prospective phase II multicenter MCL-001 “EMERGE” study led to FDA approval of lenalidomide for patients with relapsed/refractory MCL after 2 prior treatments, that included bortezomib. The activity of lenalidomide was seen regardless of MIPI, number of prior therapies, prior high dose therapy, bulky disease or high tumor burden. One of the most established prognostic factors in MCL is the proliferation index Ki67 (MIB1), now confirmed both in standard and dose-intensive high-dose therapy strategies. We present here longer follow-up of efficacy and safety from the MCL-001 study in patients relapsed/refractory to bortezomib and the potential relationship between Ki-67 and efficacy outcomes. Methods Patients with heavily pretreated MCL, that included prior bortezomib, received lenalidomide 25 mg/day PO, days 1-21 in 28-day cycles until disease progression or intolerability. Primary study endpoints were overall response rate (ORR) and duration of response (DOR); secondary endpoints included complete response (CR), time to response (TTR), progression-free survival (PFS), overall survival (OS), and safety. Response rates and time-to-event data were analyzed by independent central reviewers per modified IWG criteria and Kaplan-Meier estimates respectively (data cut-off March 20, 2013). Ki-67 was examined as an exploratory endpoint by immunohistochemistry for 81/134 patients (60%) either performed on biopsy samples for 24 patients, or based on the Ki-67 scores reported in local pathology reports for 57 patients. Results Median age for the enrolled intent-to-treat patient population (N=134) was 67 years (range, 43-83; 63% ≥65 years). The median number of previous therapies was 4 (range, 2-10; 78% received ≥3), 93% stage III/IV, and 72% were <6 months from last prior treatment. At a median follow-up of 13.2 months, the ORR was 28% (CR/CRu 8%), with a median DOR of 16.6 months (95% CI, 9.2-26.7; median not reached in patients with CR/CRu) by central review. Median TTR was 2.3 months (95% CI, 1.7-13.1), with a median time to CR/CRu of 4.1 months (95% CI, 1.9-13.2). Median PFS was 4.0 months (95% CI, 3.6-6.9), and median OS was 20.9 months (95% CI, 13.7-24.4). The average dose intensity of lenalidomide was 20 mg/day, for a median duration of 94.5 days (range, 1-1,256). Dose reductions or interruptions due to adverse events (AEs) occurred in 40% and 58% of patients, respectively. Neutropenia (44%), thrombocytopenia (28%), and anemia (11%) were the most common treatment-related grade 3/4 AEs. Ki-67 results were available in 81/134 patients, and efficacy data were categorized using 30% and 50% cut-off thresholds for Ki-67 expression (Table 1). Although patient numbers were limited, the ORR was similar in both lower and higher Ki-67 group, but those with lower Ki-67 levels demonstrated better CR rates, DOR and survival outcomes compared with patients with elevated Ki-67. Conclusions Single-agent lenalidomide in heavily pretreated patients with relapsed/refractory MCL post-bortezomib showed durable long-term efficacy with a consistent safety profile. Consistent with what is reported in the literature, high Ki-67 is associated with poor outcome in our cohort with shorter OS. Though based on retrospective evaluation and subsets of patients, the ORR to lenalidomide was similar in both low and high Ki-67 groups, suggesting lenalidomide can be active in patients expressing high levels of Ki67. Prospective studies are needed to confirm these findings. Disclosures: Goy: Celgene: Consultancy, Research Funding, Speakers Bureau. Off Label Use: This is a phase 2 clinical study of safety and efficacy for lenalidomide in patients with MCL. Williams:Celgene: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding. Drach:Celgene: Honoraria. Ramchandren:Celgene: Research Funding. Zhang:Celgene: Employment. Cicero:Celgene: Employment. Fu:Celgene: Employment. Heise:Celgene: Employment, Equity Ownership. Witzig:Celgene: Research Funding.

Burkitt Transformation of Mantle Cell Lymphoma.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4129-4129
Author(s):  
Kavita S. Reddy ◽  
Mohammad Ansari-Lari ◽  
Bruce Dipasquale
Keyword(s):  
Bone Marrow ◽  
Mantle Cell Lymphoma ◽  
Peripheral Blood ◽  
Blood Smear ◽  
Cell Lymphoma ◽  
Peripheral Blood Smear ◽  
Neoplastic Cells ◽  
Ki 67 ◽  
Mantle Cell ◽  
Neoplastic Lymphocytes

Abstract MYC rearrangements are not included as a genetic change in the blastoid variants of mantle cell lymphoma (Jaffe, et al (2001) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press.). We present two cases both with CCND1/IGH and MYC rearrangements. Case 1. An 82-year-old male with no known history of lymphoma presented with thrombocytopenia, loss of appetite and “abdominal fullness.” Imaging studies showed enlarged retroperitoneal lymph nodes. The peripheral blood smear had 18,000 WBC with approximately 30% circulating atypical lymphocytes. Flow cytometric studies of the bone marrow revealed a surface kappa light chain restricted CD10+ B-cell population. A bone marrow biopsy showed >90% of marrow cellularity comprised of neoplastic lymphocytes. The neoplastic lymphocytes were small to intermediate in size with minimal amounts of dark blue cytoplasm and several cytoplasmic vacuoles (Burkitt like morphology). By immunohistochemical stains, the neoplastic cells were positive for CD20, CD43, CD10, BCL-6, and cyclin D1, weakly and focally positive for BCL-2, and negative for CD23. The Ki-67 proliferation fraction was ∼100%. An immunohistochemical stain for CD5 was predominantly negative with a possible very faint blush on a subset of neoplastic B-cells. The FISH tests on bone marrow interphases were positive for a CCND1/IGH, a variant MYC/IGH, a variant MYC-BA rearrangements and negative for BCL6-BA and BCL2/IGH rearrangements. The variant MYC/IGH pattern was 3xMYC, 3xIGH, 1xFusion signals and MYC-BA pattern was 2x5′MYCcon3′MYC, 1x3′MYC. rearrangements. The karyotype was 44∼45,XY,del(2)(q11.2q21),der(3;17)(p10;q10), der(5)t(3;5)(q12;q15), t(11;14) (q13;q32) [cp6]/46,XY[14]. Since the karyotype had a t(11;14) and two normal 8 chromosomes, a metaphase FISH was analyzed to localize the signals for the MYC/IGH probe. The MYC signal were on both normal 8 chromosomes, a fusion signal was on a F-G sized chromosome. While the IGH signals were on the normal 14, der(14) and der(11). This was consistent with a cryptic MYC/IGH fusion in a three way rearrangement between chromosomes 8, 11 and 14. Case 2. A 69-year-old male having had a kidney transplant in 2001 was on immunosuppressive therapy. He presented with severe leukocytosis, anemia and thrombocytopenia and weight loss of about 12 pounds over several months. A peripheral blood smear showed 74,000 WBC with approximately 30% blasts. Bone marrow biopsies revealed normocellular bone marrow (50% cellularity). Interspersed large neoplastic lymphoid cells were shown by immunohistochemical stains to be positive for CD20, BCL-1, weak positive for BCL-2 and a Ki-67 staining > 90%. Flow cytometry indicated that the neoplastic cells were positive for kappa and CD5 but negative for CD11c and CD23. Interphases FISH on peripheral blood was positive for a CCND1/IGH rearrangement. The karyotype was 42∼44,X,-Y,add(1)(p13), t(2;8)(p12;q24), der(2)t(2;15)(p25;q11.2),+3,del(9)(p22p24),+del(9)(p22p24), − 10, del(11)(q21q23), t(11;14)(q13;q32) , − 13, − 15, − 17,add(17)(p11.2)[cp7]/46,XY[17]. FISH confirmed a MYC rearrangement. Therefore, this case had both CCND1/IGH and MYC/IGK rearrangement. Concomitant occurrence of a CCND1/IGH and a MYC rearrangement is rare in lymphomas. In Mitelman database of chromosome aberrations in cancer 2007, Four cases had both a t(11;14) and a t(8;14) translocation and two cases had both a t(11;14) and a t(2;8) translocation. This study expands the repertoire of abnormalities seen in blastoid transformation of mantle cell lymphoma. Being cognizant of a possible MYC involvement in the transformation of mantle cell lymphoma and its exploration would influence therapy.

scholarly journals Mantle Cell Lymphoma: A Clinicopathologic Study of 80 Cases

Blood ◽  
1997 ◽  
Vol 89 (6) ◽  
pp. 2067-2078 ◽  
Author(s):  
Larry H. Argatoff ◽  
Joseph M. Connors ◽  
Richard J. Klasa ◽  
Douglas E. Horsman ◽  
Randy D. Gascoyne
Keyword(s):  
Prognostic Factors ◽  
Mantle Cell Lymphoma ◽  
Mitotic Activity ◽  
Peripheral Blood ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Bone Marrow Involvement ◽  
Distinct Type ◽  
Mantle Cell ◽  
Peripheral Blood Involvement

Abstract Mantle cell lymphoma (MCL) is a relatively uncommon yet distinct type of malignant lymphoma whose clinical and pathological characterization has been limited by the small numbers of cases published to date. We studied 80 cases of MCL seen at a single institution over 7 years to determine both clinical and pathological prognostic factors. The patients in this study were predominantly male (70%) and older (mean age, 63 years) and presented with advanced-stage disease (88%). Extranodal involvement was common. Median overall survival (OS) was 43 months. Except for performance status, prognosis was not significantly influenced by clinical prognostic factors. Histologically, MCL architecture was classified as diffuse (78%), nodular (16%), or mantle zone (6%); the OS among these groups was identical. Increased mitotic activity (<20 mitotic figures per 10 high power fields), blastic transformation, and peripheral blood involvement at diagnosis also predicted for a worse outcome, but bone marrow involvement did not. The presence or absence of a translocation t(11; 14) by cytogenetic analysis or a bcl-1 rearrangement by Southern analysis did not significantly predict outcome. In summary, this study of 80 cases of MCL highlights its distinctive clinicopathologic features and shows that increased mitotic activity, blastic morphology, and peripheral blood involvement at diagnosis are prognostically important factors.

Rituximab Plus Gemcitabine and Oxaliplatin as Salvage Therapy In Patients with Relapsed/Refractory Mantle-Cell Lymphoma.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2825-2825 ◽  
Author(s):  
Mercedes Gironella ◽  
Andrés López, MD ◽  
Abrisqueta Pau ◽  
Jaramillo Anny ◽  
Purroy Noelia ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Clinical Stage ◽  
Performance Status ◽  
Median Number ◽  
Significant Activity ◽  
Mantle Cell ◽  
Grade 3

Abstract Abstract 2825 Mantle cell lymphoma (MCL) constitutes one of the lymphomas with poorest prognosis at relapse and there are no effective salvage. The activity shown by the combination Gemcitabine and Oxaliplatin in several types of limfoma, along with its “in vitro” synergistic effect, made this regimen an attractive regimen for salvaging patients with MCL. Against this background, we performed an off-label pilot study in order to assess efficacy and toxicity of the combination R-GemOx in relapsed or refractory patients with MCL. For this, 27 patients (70% male, median age 70 years) diagnosed with MCL between November 2004 and January 2010 were included in this study. Inclusion criteria were adequate performance status, confirmed diagnosis of MCL and relapse or refractoriness to the previous treatment. The regimen consisted of Rituximab 375 mg/m2 on day 1, Gemcitabine 1000 mg/m2 and Oxaliplatin 100 mg/m2 on day 2, every 14 days, up to 8 cycles. Dose and interval were adjusted according to hematological and extrahematological toxicities. Median number of previous regimens was 1 (range 1 to 3), being EPOCH-R (n=14), R-CHOP (n=6), and RFC (n=3) the most frequently used induction therapies. Twelve patients relapsed after prior CR, 10 progressed after achieving a PR, whereas 5 were refractory to therapy. At inclusion, 85% of patients were in advanced (III/IV) clinical stage, 40% had bone marrow infiltration, 28% gastrointestinal involvement, and 17% cavum infiltration. Median number of cycles administered was 8 (range, 3 to 8). Doses were reduced in 9 cycles and delayed in 15 cycles. Neutropenia grade 3–4 was observed in 9 cycles and thrombocytopenia grade 3–4 in 6. Hepatotoxicity grade 1–2 in 5 pts and grade 3 in 1, sensitive neurotoxicity grade 1–2 in 12 pts, and renal impairment grade 2 in 1 patient. After completion of the treatment, 21 pts (77%) were considered in CR/uCR, 1 (4%) achieved a PR, 1 a SD, whereas 4 PD. Six patients subsequently received a stem-cell transplantation (4 allogeneic, 2 autologous). Thirteen pts are still alive and out of progression. Ten pts have died (8 due to progression and 2 due to acute GVHD). With a median follow-up of 23 months (range: 3–57), PFS and OS at 2 years are 41% and 58%, respectively. The R-GemOx combination showed a significant activity in relapsed or refractory pts with MCL with a very acceptable toxicity profile. These results prompted us to conduct a multicenter phase II clinical trial that is now ongoing. Disclosures: López: Roche Farma: Research Funding, Travel Support. Bosch:Roche Farma: Research Funding.

Ibrutinib and Rituximab Are an Efficacious and Safe Combination in Relapsed Mantle Cell Lymphoma: Preliminary Results from a Phase II Clinical Trial

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 627-627 ◽  
Author(s):  
Michael (Luhua) Wang ◽  
Fredrick Hagemeister ◽  
Jason R. Westin ◽  
Luis Fayad ◽  
Felipe Samaniego ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Mantle Cell Lymphoma ◽  
Phase Ii ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Single Agent ◽  
Phase Ii Clinical Trial ◽  
Hematologic Toxicity ◽  
Ki 67 ◽  
Mantle Cell

Abstract Single-agent ibrutinib has been approved by the FDA for patients with mantle cell lymphoma (MCL) who received at least one prior therapy based on a phase II clinical trial in which ibrutinib elicited a response rate of 68% (Wang et al, NEJM, 2013). In this clinical study we found a transient increase in circulating MCL lymphocytes during the initial phase of tumor reduction. We hypothesized that targeting the circulating MCL cells with intravenous rituximab will further improve the efficacy of ibrutinib. We conducted a single-center phase II clinical trial with ibrutinib in combination with rituximab for relapsed MCL with no upper limit for prior lines of therapy. Among 50 patients with MCL, 100% received prior rituximab, 77% received prior Hyper-CVAD, 75% received prior bortezomib, and 20% received prior lenalidomide. Rituximab was dosed at 375 mg/m2 iv weekly X 4 during cycle 1 (cycle = 28 days), then on day 1 of every cycle from 3-8, and thereafter once every other cycle up to 2 years. Ibrutinib was dosed at 560 mg orally daily continuously. With a median follow up time of 6.5 months (range 1-10), 45 patients are evaluable for toxicity and efficacy as of July 21, 2014. Thirty three patients (73% of evaluable patients) have Ki-67 < 50%. Seventeen (17) patients are now off study including 2 patients with secondary malignancies (AML and lung cancer). One (1) patient in CR withdrew consent due to social issues and continued on commercial ibrutinib. Two (2) patients in remission withdrew consent due to their concerns that rituximab-ibrutinib might worsen their atrial fibrillation and both continued on single-agent commercial ibrutinib. One patient was off study due to bleeding. Three (3) patients in remission went off to stem cell transplantation. Eight (8) patients are off study due to progressive MCL (4 never responded: 4 responded then progressed), all of them had Ki-67 greater than 50% (range 50-100%). There were no toxic deaths due to therapy. Grade 3 hematologic toxicity events included neutropenia (1) and thrombocytopenia (1). The most common (≥ 20%) grade 1-2 non-hematologic toxicity events regardless of its relationship with study therapy included fatigue (18), diarrhea (11), myalgia (11), dyspnea (11), blurred vision (10), nausea (9),dry eye (9) and atrial filbrillation (6). The efficacy data is listed in Table 1. The ORR to date is 87% with CR in 17 patients (38%) and PR in 22 patients (49%). The CR rate is high in this study in the context of historical data (21% by single-agent ibrutinib). Median duration of response and PFS has not been reached. Notably, all 10 patients with SD (2) and PD (8) have Ki-67’s ≥ 50%. Excluding the 12 out of 45 evaluable patients with Ki-67 ≥ 50%, the ORR for 33 patients with lower Ki-67 (< 50%) is 100% (48% for CR and 52% for PR) in patients with relapsed/refractory MCL. While this trial is ongoing, preliminary data indicated that Ibrutinib-rituximab combination is well-tolerated and is efficacious, especially in patients with Ki-67 less than 50%. Table 1 The best response related to Ki-67 All n (%) Ki-67 < 50% Ki-67 ≥ 50% Evaluable patients 45 33 12 ORR 39 (87%) 33 (100%) 6 (50%) CR 17 (38%) 16 (48%) 1 (8%) PR 22 (49%) 17 (52%) 5 (42%) SD 2 (4%) 0 2 (17%) PD 4 (9%) 0 4 (33%) Duration of response NR NR NR PFS NR NR NR Disclosures Wang: Pharmacyclics and Janssen: Honoraria, Research Funding. Off Label Use: Ibrutinib and Rituximab for mantle cell lymphoma clinical trial. Westin:Pharmaciclics and Janssen: Honoraria, Research Funding. Fayad:Pharmacyclics and Janssen: Research Funding. Samaniego:Pharmacyclics and Janssen: Research Funding. Romaguera:Pharmacyclics and Janssen: Research Funding.

scholarly journals Clinical characteristics and outcomes of primary versus secondary gastrointestinal mantle cell lymphoma

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Alessia Castellino ◽  
Aung M. Tun ◽  
Yucai Wang ◽  
Thomas M. Habermann ◽  
Rebecca L. King ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Cell Lymphoma ◽  
Performance Status ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Local Therapy ◽  
Progression Free Survival ◽  
Cell Transplant ◽  
Multiple Lesions ◽  
Mantle Cell

AbstractPrimary gastrointestinal (GI) mantle cell lymphoma (MCL) is rare and the optimal management is unknown. We reviewed 800 newly diagnosed MCL cases and found 22 primary (2.8%) and 79 (9.9%) secondary GI MCL cases. Age, sex, and performance status were similar between primary and secondary cases. Secondary cases had more elevations in lactate dehydrogenase (28% vs 0%, P = 0.03) and a trend for a higher MCL international prognostic index (P = 0.07). Observation or local therapy was more common for primary GI MCL (29% vs 8%, P < 0.01), and autologous stem-cell transplant was more common for secondary GI MCL (35% vs 14%, P < 0.05). The median follow-up was 85 months. Primary and secondary GI MCL had similar 5-year progression-free survival (PFS) (30% vs 28%, P = 0.59) and overall survival (OS) (65% vs 66%, P = 0.83). The extent of GI involvement in primary GI MCL affected treatment selection but not outcome, with a 5-year PFS of 43% vs 14% vs 31% (P = 0.48) and OS of 57% vs 71% vs 69% (P = 0.54) in cases with single lesion vs multiple lesions in 1 organ vs multiple lesions in ≥2 organs. Less aggressive frontline treatment for primary GI MCL is reasonable. It is unknown whether more aggressive treatment can result in improved outcomes.

Leukemic Variant of Mantle Cell Lymphoma: Clinical Presentation and Management

2021 ◽  
Vol 23 (9) ◽  
Author(s):  
Krista M. Isaac ◽  
Craig A. Portell ◽  
Michael E. Williams
Keyword(s):  
Mantle Cell Lymphoma ◽  
Clinical Presentation ◽  
Cell Lymphoma ◽  
Mantle Cell

scholarly journals High-dose cytarabine does not overcome the adverse prognostic value of CDKN2A and TP53 deletions in mantle cell lymphoma

Blood ◽  
2015 ◽  
Vol 126 (5) ◽  
pp. 604-611 ◽  
Author(s):  
Marie-Hélène Delfau-Larue ◽  
Wolfram Klapper ◽  
Françoise Berger ◽  
Fabrice Jardin ◽  
Josette Briere ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Prognostic Value ◽  
Cell Lymphoma ◽  
Standard Of Care ◽  
High Dose ◽  
Current Standard ◽  
Ki 67 ◽  
Key Points ◽  
Mantle Cell ◽  
High Dose Cytarabine

Key Points CDKN2A and TP53 deletions remain of bad prognostic value in younger MCL patients treated according to the current standard of care. CDKN2A and TP53 deletions have independent deleterious effects and should be considered for treatment decisions in addition to MIPI and Ki-67 index.

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