Lymphoma

Paediatric type follicular lymphoma (PTFL) is an uncommon nodal follicular lymphoma (FL) that occurs primarily in children and young adults, but also occurs sporadically in older individuals.

Morphologically :

• Architecture: 

• At least partial effacement of nodal architecture (required) 

• Pure follicular proliferation (required)

• Expansile follicles

• Intermediate-sized so-called blastoid cells (not centrocytes)

• Immunohistochemistry:

• BCL6 positivity

• BCL2 negativity or weak positivity 

• High proliferative fraction (>30%)

• Genomic:(required)

• No BCL2, BCL6, IRF4, or aberrant IG rearrangement 

• No BCL2amplification

• Clinical features:

• Nodal disease (required) Stage I-IIdisease (required) 

• Patient age <40 years

• Marked male predominance  

Managment:

• Tumor is too rare to conduct prospective trials

• Chemo regimens primarily based on  NHL BFM protocols, which are likely to be anthracycline-based (R-CHOP-like) for cases where complete resection is not achieved. 

• Conservative approach should be considered in cases with an entirely resectable lesion to avoid the long-term effects of chemotherapy in younger populations.

References:

1. Alnughmush A, Fakih RE, Mohammed S, Aljurf M. Pediatric-type follicular lymphoma: a short review. Int J Hematol Oncol. 2022 Nov 22;11(4):IJH41. doi: 10.2217/ijh-2022-0003. PMID: 36514787; PMCID: PMC9732916.

2. WHO Hematolymphoid system 2016