Clinical characteristics and prognostic features of patients with blastoid and pleomorphic variant mantle cell lymphoma: a real-world study in China

中国真实世界研究:母细胞样和多形性变异型套细胞淋巴瘤患者的临床特征和预后因素

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Abstract

Blastoid and pleomorphic mantle cell lymphoma (B/P-MCL) represents a high-risk subtype of hematological malignancy with an unfavorable prognosis. We conducted a retrospective analysis of 76 patients with classic MCL and 81 patients with B/P-MCL between January 2010 and May 2024 to investigate clinical characteristics, outcomes, and prognostic factors. Ki-67 ≥ 30%, high MCL International Prognostic Index (MIPI) scores, and frequent CD5 negativity were more prevalent in B/P-MCL than in classic MCL. In B/P-MCL, TP53 mutations (46.2%) were most common, followed by ATM (42.3%), CCND1 (23.1%), NOTCH1 (23.1%), KMT2D (23.1%), SMARCA4 (15.4%), and BIRC3 (15.4%). Multivariate analysis revealed that Ki−67% ≥90% and bone marrow involvement were independent prognostic factors for overall survival (OS); Ki−67% ≥90% and splenic involvement were independent prognostic factors for progression-free survival (PFS). The blastoid and pleomorphic variants of MCL display distinct morphological characteristics and exhibit certain clinical differences; however, no statistically significant differences in PFS or OS have been demonstrated between these two subtypes. B/P-MCL is characterized by highly aggressive features and a dismal clinical course. Even with the adoption of intensified treatment protocols, the long-term prognosis for these patients remains unsatisfactory. Strengthened efforts to explore novel therapeutic strategies are crucial and may ultimately translate into improved clinical outcomes for this patient population.

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