MYC/BCL2 double hit lymphoma (DHL) has been the subject of many studies; however, no study has systemically compared the clinicopathologic features and prognostic factors between patients with de novo disease versus those with a history of follicular lymphoma (FL). In addition, the prognostic importance of several other issues remains controversial in these patients. In this retrospective study, we assess 157 patients with MYC/BCL2 DHL including 108 patients with de novo disease and 49 patients with a history of FL or rarely other types of low-grade B-cell lymphoma. Patients received induction chemotherapy regimens including 61 R-CHOP, 31 R-EPOCH, 29 R-Hyper-CVAD, and 23 other regimens. Thirty-nine patients received a stem cell transplant (SCT) including 31 autologous and 8 allogeneic. Sixty-two patients achieved complete remission (CR) after induction chemotherapy. Median overall survival (OS) was 19 months. Clinicopathologic features were similar between patients with de novo tumors versus those with a history of FL (P > 0.05). Using multivariate analysis, achieving CR, undergoing SCT, stage and the International Prognostic Index were independent prognostic factors for OS. Stem cell transplantion was associated with improved OS in patients who failed to achieve CR, but not in patients who achieved CR after induction chemotherapy. In conclusion, patients with MYC/BCL2 DHL who present with de novo disease and patients with a history of FL have a similarly poor prognosis. Achievement of CR, regardless of the induction chemotherapy regimen used, is the most important independent prognostic factor. Patients who do not achieve CR after induction chemotherapy may benefit from SCT.
Mantle cell lymphoma is usually negative for CD10 which is useful in distinguishing MCL from other CD10 + B cell lymphomas. Here we assessed the clinicopathologic features of 30 cases of CD10+ MCL, the largest series to date in the English literature, and compared them with a group of 212 typical MCL cases (CD5+, CD10-negative, CD23-negative, cyclin D1+). The 30 patients with CD10+ MCL included 17 men and 13 women with a median age of 68 years. Compared with CD10-negative MCL, patients with CD10+ MCL showed a lower male predominance (p = 0.01), more often had a diffuse growth pattern (p = 0.04) and blastoid/pleomorphic morphology (p < 0.0001), and more often showed BCL6 expression (p = 0.009). In all MCL patients, CD10 expression was not associated with overall survival (OS) (p = 0.16). However, in more aggressive subsets of MCL patients including those with high Ki67 (> 60%), blastoid/pleomorphic morphology, or high MCL International Prognostic Index (MIPI), CD10 expression was associated with a worse OS (p = 0.003, 0.04, and 0.001, respectively). High Ki67 (> 60%), blastoid/pleomorphic morphology, and high MIPI were also been identified as poor prognostic factors patients with in CD10+ MCL (p = 0.001, 0.0003, and 0.01, respectively). In summary, CD10+ MCL more often has a diffuse growth pattern, blastoid/pleomorphic morphology, and BCL6 expression. In MCL patients with a high Ki-67 (> 60%), blastoid/pleomorphic morphology, or high MIPI, CD10 expression contributes to an even worse prognosis. MCL should be included in the differential diagnosis of CD10 + B cell lymphomas.
Mantle cell lymphoma (MCL) represents 4% to 9% of all non-Hodgkin lymphomas and is characterized by CD5 and cyclin D1 expression and t(11;14)(q13;q32). However, about 5% of MCL lack CD5 expression and is poorly characterized. Here, we present 58 patients with CD5 negative (CD5−) MCL and compared them with a group of 212 typical CD5 positive (CD5+) MCL cases. There were 39 men and 19 women with a median age of 66 years (range, 36 to 88). Compared with CD5 positive (CD5+) MCL patients, patients with CD5− MCL showed a lower male-to-female ratio (P=0.006) and a higher frequency of “bone marrow non-nodal” presentation (P=0.01). All other clinicopathologic features, including the frequency of SOX11 expression, were similar between the 2 groups. Treated with similar regimens, patients with CD5− MCL showed a significantly longer progression-free survival (PFS) (P=0.01) and a tendency for longer overall survival (OS; P=0.078) than CD5 positive (CD5+) MCL patients. Univariate analysis showed of the well-known poor prognostic factors, only Mantle Cell Lymphoma International Prognostic Index was an inferior prognostic factor and blastoid/pleomorphic morphology and high Ki67 were not associated with prognosis in CD5− MCL patients. Multivariate Cox regression analysis showed CD5 expression was an independent prognostic factor for PFS (P=0.031) but not OS in MCL patients. In conclusion, the results suggest that patients with CD5− MCL have a more favorable prognosis than CD5+ MCL patients, although the clinicopathologic features of both groups are largely similar. CD5− MCL may represent a distinct variant of MCL and needs to be included in the differential diagnosis of CD5− small B-cell lymphomas.
Mantle cell lymphoma (MCL) is usually CD23-negative, a feature helpful in distinguishing MCL from chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). However, a subset of MCL cases can be CD23+. Limited data are available regarding the clinicopathologic features and prognosis of patients with CD23+ MCL. In this study, we reviewed 798 cases of MCL and identified 103 (13%) that were CD23+ by flow cytometry, all of which were positive for cyclin D1 and/or associated with CCND1/IGH. In all cases of CD23+ MCL, CD23 expression was dim partial or dim, unlike moderate to bright CD23 expression observed in CLL/SLL. The clinicopathologic features and outcome of patients with CD23+ MCL were compared with 240 patients with typical MCL negative for CD23. Patients with CD23+ MCL more often had an elevated leukocyte count (33% vs 18%, p=0.009), bone marrow involvement (89% vs 78%, p=0.02), stage 4 disease (87% vs 77%, p=0.03) and a leukemic presentation (42% vs 11%, p=0.0001). CD23+ MCL was also more often positive for CD200 (17% vs. 4.6%, p=0.0005) and less commonly positive for SOX11 (55% vs. 74%, p=0.027). All other clinicopathologic features were similar. With similar treatment regimens and observation times, patients with CD23+ MCL had a significant better overall survival (p=0.02) and progression-free survival (p=0.029). In conclusion, CD23 expression was observed in 13% of MCL cases and is associated with a better prognosis in patients with MCL. CD23 is associated with leukocytosis, a leukemic presentation, bone marrow involvement, CD200 expression, and a lower frequency of SOX11 positivity.
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