images in haematology
Paediatric follicular lymphoma
A 15-year-old male presented with a 2-month history of swelling in the submental area (top panels). He was otherwise asymptomatic and no other abnormality was found on examination. Magnetic resonance imaging demonstrated a clearly defined mass in the submental area. An excision biopsy was performed and the histological appearance supplemented by immunohistochemistry was diagnostic of follicular lymphoma (FL). Multiplex polymerase chain reaction demonstrated clonal IGH (VFR1-J, VFR2-J and VFR3-J) and IGK light chain (V-J) gene rearrangement; however the t(14;18) BCL2-JH chromosomal translocation was absent. Further imaging, including positron emission tomography, was negative and a bone marrow trephine biopsy did not show involvement. A final diagnosis of paediatric follicular lymphoma was made. As the disease was stage I, following complete resection we decided on a ‘watch and wait’ strategy for this patient. He remains well at 4 years from diagnosis.
ª 2013 John Wiley & Sons Ltd British Journal of Haematology, 2013, 163, 1
Paediatric FL is rare and usually involves cervical lymph nodes, Waldeyer ring and extranodal sites. It is considered a variant of FL that is associated with male gender (3:1), teenage years, low serum lactate dehydrogenase level, grade 3 histology (bottom left) and limited stage disease (I/II). Paediatric FL is genetically distinct from adult FL in that IRF4 translocations are far more common, while the translocation t(14;18)(q32;q21) affecting BCL2 gene expression, present in 85% of adult cases, is rarely seen in paediatric cases (bottom right). The overall outcome of paediatric FL is excellent. Johnny McHugh,1 Lorna Storey,1 Larry Bacon,1 Aengus O’Marcaigh,1,3 Maureen O’Sullivan2 and Owen Smith1,3 1 Departments of Haematology, 2Histopathology, Our Lady’s Children’s Hospital, Crumlin, and 3Trinity College Dublin, Dublin, Ireland. E-mail:
[email protected]
First published online 8 August 2013 doi:10.1111/bjh.12493
