Diffuse large B-cell lymphomas with plasmablastic/plasmacytoid features are associated with TP53 deletions and poor clinical outcome

Leukemia. 2004 Jan;18(1):146-55. doi: 10.1038/sj.leu.2403206.

Abstract

To define reproducible criteria for subgroups of diffuse large B-cell lymphomas (DLBCL), including lymphomas with plasmablastic/plasmacytoid features (PB/PC-Fs), we investigated 66 DLBCL; the samples were categorized as either centroblastic (CB), immunoblastic (IB) or PB/PC-F applying standardized morphologic criteria. Blinded specimens were reviewed by three independent pathologists. The final consensus classification included 44 CB (67%), seven IB (10%) and 15 PB/PC-F (23%). The interobserver agreement between two centers (Vienna, Würzburg) was 93.5%. Most PB/PC-F were CD20+, cIgM+, MUM-1+, CD138+/-, bcl-6-, corresponding to an activated B-cell phenotype. Immunoglobulin-V(H) gene mutation analysis was consistent with a germinal or postgerminal center-cell origin. By fluorescence in situ hybridization analysis, 11/13 (85%) PB/PC-F had a monoallelic TP53 deletion. The pretreatment characteristics of patients with PB/PC-F included a tendency for more B symptoms, extranodal disease and a higher IPI. Importantly, PB/PC-F were resistant to standard chemotherapy (complete remission rate 47%, relapse rate 71%) and even autologous stem-cell transplantation. The median overall survival (OS) (14 months, P<0.002) and disease-free survival (6 months, P=0.02) were significantly shorter compared to patients with CB and IB. The OS difference was pronounced within the low and low-intermediate IPI risk group (P<0.001). Our data indicate a strong association of plasmablastic/plasmacytoid morphology with TP53 deletions, poor response to chemotherapy and short survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, Neoplasm / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers / analysis
  • Female
  • Follow-Up Studies
  • Genes, Immunoglobulin
  • Genes, p53 / genetics*
  • Germinal Center / immunology
  • Herpesvirus 4, Human / genetics
  • Humans
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Lymphoma, B-Cell / classification
  • Lymphoma, B-Cell / mortality
  • Lymphoma, B-Cell / pathology*
  • Lymphoma, Large B-Cell, Diffuse / classification*
  • Lymphoma, Large B-Cell, Diffuse / mortality
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Large-Cell, Immunoblastic / classification
  • Lymphoma, Large-Cell, Immunoblastic / mortality
  • Lymphoma, Large-Cell, Immunoblastic / pathology
  • Male
  • Middle Aged
  • Plasma Cells / pathology*
  • Prognosis
  • RNA, Viral / genetics
  • Sequence Deletion
  • Survival Rate
  • Treatment Outcome

Substances

  • Antigens, Neoplasm
  • Biomarkers
  • RNA, Viral