Journal article

Publication year: 2010

Pages: 253-259

Journal: Haematologica

Volume number: 95

Issue number: 2

ISSN: 0390-6078

eISSN: 1592-8721

Open access status: Gold

DOI Link: https://doi.org/10.3324/haematol.2009.013177

Publisher: Ferrata Storti Foundation

Abstract: 

Background

Pediatric follicular lymphoma has recently been recognized as a novel variant of follicular lymphoma in the World Health Organization classification of lymphomas. Given the rarity of the disease, histopathological and genetic data on this type of lymphoma are still scarce.

Design and Methods

We analyzed 25 cases of pediatric follicular lymphoma (patients aged :518 years) by morphology, immunohistochemistry and interphase fluorescence in situ hybridization. All patients analyzed were treated within Non-Hodgkin's Lymphoma - Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials, and the cohort was representative of the German population.

Results

The genetic hallmark of adult follicular lymphoma, t(14;18)(q32,q21), was not detectable in any of the pediatric cases, although BCL2 protein was expressed in 55%, of the latter cases. No correlation was found between BCL2 protein expression and outcome. Chromosomal breaks in the immunoglobulin heavy chain gene (IGH) and the BCL6 locus were detected in 5 of 17 and I of 18 cases, respectively. Patients with pediatric follicular lymphoma had long event-free survival and, in contrast to adult Follicular lymphoma, the clinical course was not dominated by relapses. A simultaneous diffuse large B-cell lymphoma was frequently detected at initial diagnosis in children but did not indicate an aggressive clinical course.

Conclusions

Our data suggest that pediatric follicular lymphoma is a disease that differs from its adult counterpart both genetically and clinically.

Harvard Citation style: Oschlies, I., Salaverria, I., Mahn, F., Meinhardt, A., Zimmermann, M., Woessmann, W., et al. (2010) Pediatric follicular lymphoma - a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin's Lymphoma - Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials, Haematologica, 95(2), pp. 253-259. https://doi.org/10.3324/haematol.2009.013177

APA Citation style: Oschlies, I., Salaverria, I., Mahn, F., Meinhardt, A., Zimmermann, M., Woessmann, W., Burkhardt, B., Gesk, S., Krams, M., Reiter, A., Siebert, R., & Klapper, W. (2010). Pediatric follicular lymphoma - a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin's Lymphoma - Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials. Haematologica. 95(2), 253-259. https://doi.org/10.3324/haematol.2009.013177