<p>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, <i>TP53</i> mutations (46.2%) were most common, followed by <i>ATM</i> (42.3%), <i>CCND1</i> (23.1%), <i>NOTCH1</i> (23.1%), <i>KMT2D</i> (23.1%), <i>SMARCA4</i> (15.4%), and <i>BIRC3</i> (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.</p>

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

  • Ying Huang,
  • Xiang Zhang,
  • Yunfei Lv,
  • Juying Wei,
  • Xingnong Ye,
  • Xuewu Zhang,
  • Chunmei Yang,
  • Yanan Zhu,
  • Jinhan Wang,
  • Gaixiang Xu,
  • Min Yang,
  • Haitao Meng,
  • Wanzhuo Xie,
  • Jie Jin,
  • Hongyan Tong,
  • Wenjuan Yu

摘要

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.