MabThera.com

BL is a highly aggressive form of NHL with rapid tumour growth leading to death within a few weeks if left untreated. It was first recognised as an endemic disorder of childhood, associated with Epstein-Barr virus infection, with the non-endemic form identified subsequently. The endemic and non-endemic forms of BL present differently, but display similar chromosomal abnormalities, primarily rearrangements of the MYC gene. In the endemic form, involvement of the jaw is common, particularly in younger children and abdominal disease is present in approximately 50% of patients at diagnosis. The non-endemic or sporadic form frequently presents with disease centred around the abdomen (approximately 90% of cases) with bone marrow involvement more common than previously thought (20%).

BL tumour cells are characteristically medium sized, monomorphic cells, with a round nucleus, multiple nucleoli and relatively abundant cytoplasm. The cells express the B-cell antigens CD19, CD20 and CD79a, and CD10, but do not express CD5 or CD23¹.

References

1. Magrath I, et al. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004:477-501.