Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centers

Abstract

We report a case of angioimmunoblastic T-cell lymphoma (AITL) in a 48-year-old Japanese male. Characteristic clinical manifestations of AITL such as fever, weight loss, skin rash, general lymphadenopathy, Coombs test positivity, and polyclonal hypergammaglobulinemia were present. Histopathologically, the nodal architecture was preserved and germinal centers were, if anything, hyperplastic, which was unusual for AITL. Diagnostic clear cells were absent. Intrafollicular tingiblebody macrophages were markedly increased. In the interfollicular zone, proliferation of UCHL-1+ immunoblasts was evident, and high-endothelial postcapillary venule-type vessels were increased. The T-cell receptor gene (Cβ1) was clonally rearranged. Based on these findings, a diagnosis of AITL with hyperplastic germinal centers was made. Six courses of CHOP were administered and no signs of relapse were noticed about two years after diagnosis.