Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Plasmablastic lymphoma (PBL) is a rare, aggressive B-cell lymphoma with limited known etiology and features.
• PBL is characterized by a poor prognosis, necessitating further research into its clinical and molecular aspects.

Purpose of the Study:

• To investigate the clinicopathologic characteristics of PBL patients in a Chinese population.
• To analyze therapeutic approaches and clinical outcomes for PBL.
• To explore molecular differences between PBL and diffuse large B-cell lymphoma (DLBCL) using RNA sequencing.

Main Methods:

• A multicenter retrospective study involving 102 PBL patients.
• Analysis of clinicopathologic features and outcomes for 56 patients with treatment and follow-up data.
• RNA sequencing performed on 6 PBL samples and 11 DLBCL samples.

Main Results:

• PBL predominantly affected immunocompetent males, with most presenting at early Ann Arbor stages (I/II).
• Lymph nodes and gastrointestinal tract were common sites of involvement.
• Complete remission (CR) post-treatment was the sole significant prognostic factor for overall survival (OS) and progression-free survival (PFS).
• RNA sequencing revealed significant downregulation of B-cell receptor (BCR), T-cell receptor (TCR), P53, calcium, and Wnt signaling pathways in PBL compared to DLBCL.

Conclusions:

• PBL in the Chinese population typically occurs in immunocompetent individuals with early-stage disease.
• Achieving CR is a critical prognostic factor for OS and PFS in PBL.
• Distinct molecular pathway differences (BCR, P53, calcium, Wnt signaling) between PBL and DLBCL offer insights into pathogenesis and potential therapeutic targets.