Pediatric-type Follicular Lymphoma Shows Unique Immunogenetic Features With Lack of Acquired N-glycosylation Sites.
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Pediatric-type follicular lymphoma (PTFL) is a rare germinal center (GC) derived neoplasm with clinicopathological and genetic features distinct from conventional t(14;18)-positive follicular lymphoma (FLpos), as well as adult-type FL lacking the t(14;18) translocation (FLneg). The immunogenetic characteristics of PTFL, including immunoglobulin (IG) V-region gene usage, somatic hypermutation (SHM), potential antigen selection and presence of acquired N-glycosylation motifs in rearranged IG genes, a hallmark of FLpos, are unknown. Therefore, the immunogenetic features of 29 PTFL were studied with NGS-based clonality determination and bioinformatic analysis. Eleven cases of adult-type FLneg were used as controls. IG heavy chain (IGH) V-gene usage of PTFL was similar to normal B-cell populations with IGHV3 genes predominating (19/29 cases). Additionally, no major bias in IGKV or IGLV usage was found. SHM was identified in 27/28 (96.4%) cases in the IGHV gene, and 20/24 (83%) of the IGKV and IGLV sequences. Ongoing SHM was observed in all cases with available full-length IGHV domain. Acquired N-glycosylation (N-X-S/T) motif in productive IG-V rearrangements were identified in only 2/29 (6.8%) PTFL cases, and 3/10 (33%) evaluable cases of FLneg. In PTFL, no correlation between IG gene usage, presence of SHM, and the mutational profile was observed. In conclusion, PTFL shows the hallmark immunogenetic features of GC derivation, but in contrast to t(14;18)-positive FL, lacks N-glycosylation motifs. This finding indicates a different, possibly antigen-dependent role of the expressed IG in PTFL. Whether these specific immunogenetic properties play a role in the indolent behavior of PTFL remains to be investigated.