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Lymphoma: Current concepts and difficult diagnostic disorders

(8;22) translocations are seen, involving respectively, the kappa and lambda Ig genes. There is a difference between endemic and non-endemic forms of the disease with the Ig joining region involved in the former and the switch region involved in the latter. The association with Epstein-Barr virus varies: virtually all endemic cases are associated with Epstein-Barr virus; whereas in the non-endemic it ranges from 15% in non-HIV associated to 40% in HIV-associated. The Epstein-Barr viral termini typically show a clonal homogenous pattern on Southern blotting, suggesting early infection of a single B cell. The clinical picture varies depending on the patients’ origin. Children (peak age 6) are affected in the endemic regions, but non-endemic patients are typically adults (peak age 30). The disease tends to be extranodal (except in the HIVassociated BL which is more often nodal). Endemic BL has the interesting property of involving bilateral sites (e.g. breasts and ovaries) and unusual sites (e.g. jaw). The breast involvement in puberty and pregnancy is associated with prolactin receptor expression. In the non-endemic areas, gutassociated lesions are common. There is a high cure rate in the endemic areas and it appears that prognosis relates to the bulk and stage of disease.