Nasal-type T/natural killer (NK) cell lymphoma, which often shows an angioc
entric growth pattern, is a distinct clinicopathological entity highly asso
ciated with the Epstein-Barr virus (EBV). This tumour has a characteristic
immunophenotype, whereas the cytological spectrum is broad. It is known tha
t a clonal T-cell receptor (TCR) gene rearrangement is not found in this tu
mour. However, it is still unresolved as to whether the finding of a clonal
TCR gene rearrangement excludes the diagnosis of nasal-type T/NK cell lymp
homa. We describe a case of nasal-type T/NK cell angiocentric lymphoma, EBV
-associated, and showing clonal TCR gamma gene rearrangement. The patient d
ied of sepsis 5 months after diagnosis in spite of aggressive chemotherapy.