AI Article Synopsis

  • * A study of 45 PNMZL cases revealed that 31% were confirmed as PNMZL, while 69% displayed combined histological traits of PNMZL and PTFL, characterized by a specific interfollicular B-cell component and low genomic complexity.
  • * The research suggested that PNMZL and PTFL may actually represent variations of the same disease due to similar genetic alterations and methylation profiles, proposing a new classification term: "pediatric-type

Article Abstract

Pediatric nodal marginal zone lymphoma (PNMZL) is an uncommon B-cell neoplasm affecting mainly male children and young adults. This indolent lymphoma has distinct characteristics that differ from those of conventional nodal marginal zone lymphoma (NMZL). Clinically, it exhibits overlapping features with pediatric-type follicular lymphoma (PTFL). To explore the differences between PNMZL and adult NMZL and its relationship to PTFL, a series of 45 PNMZL cases were characterized morphologically and genetically by using an integrated approach; this approach included whole-exome sequencing in a subset of cases, targeted next-generation sequencing, and copy number and DNA methylation arrays. Fourteen cases (31%) were diagnosed as PNMZL, and 31 cases (69%) showed overlapping histologic features between PNMZL and PTFL, including a minor component of residual serpiginous germinal centers reminiscent of PTFL and a dominant interfollicular B-cell component characteristic of PNMZL. All cases displayed low genomic complexity (1.2 alterations per case) with recurrent 1p36/TNFRSF14 copy number-neutral loss of heterozygosity alterations and copy number loss (11%). Similar to PTFL, the most frequently mutated genes in PNMZL were MAP2K1 (42%), TNFRSF14 (36%), and IRF8 (34%). DNA methylation analysis revealed no major differences between PTFL and PNMZL. Genetic alterations typically seen in conventional NMZL were absent in PNMZL. In summary, overlapping clinical, morphologic, and molecular findings (including low genetic complexity; recurrent alterations in MAP2K1, TNFRSF14, and IRF8; and similar methylation profiles) indicate that PNMZL and PTFL are likely part of a single disease with variation in the histologic spectrum. The term "pediatric-type follicular lymphoma with and without marginal zone differentiation" is suggested.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9631662PMC
http://dx.doi.org/10.1182/bloodadvances.2022007322DOI Listing

Publication Analysis

Top Keywords

pnmzl ptfl
12
marginal zone
12
pnmzl cases
12
pnmzl
11
ptfl
8
nodal marginal
8
zone lymphoma
8
follicular lymphoma
8
copy number
8
dna methylation
8

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!