Part of the natural history of follicle center lymphoma (FCL) is transforma
tion to a more aggressive neoplasm, almost always a diffuse large B-cell ly
mphoma. We describe a rare example of a precursor B-lymphoblastic transform
ation of grade I FCL occurring in a 45-year-old woman 12 years after initia
l presentation and 3 years after successful treatment for a diffuse large c
ell transformation. The lymphoblastic lymphoma shared the same immunoglobul
in heavy chain gene rearrangement as the FCL as assessed by polymerase chai
n reaction amplification and direct sequencing, as well as identical kappa
light chain gene rearrangements by Southern blot analysis. The immunoglobul
in heavy chain variable gene sequences of both tumors showed numerous ident
ical base substitutions compared with germline sequences and 3 additional m
utations in the lymphoblastic lymphoma not present in the low-grade FCL. Th
ese results indicate origin of the lymphoblastic process from the mature fo
llicle center B-cell clone, rather than divergent origin of the 2 tumors fr
om a common immature B-cell precursor.