Objective: To explore the clinicopathologic features and diagnosis of splenic T-cell and NK-cell neoplasms.

Methods: Nine cases of splenic T-cell and NK-cell neoplasms were collected and studied by morphology, immunophenotyping, EBER in situ hybridization and TCR-gamma gene rearrangement. Antibodies used were as follows: CD45RO, CD3epsilon, CD3, CD4, CD8, CD56, TIA-1, GranzymeB, CD30, Ki-67 and CD20.

Results: Among the 9 cases, hepatosplenic T-cell lymphoma (HSTCL) and extranodal nasal type NK/T-cell lymphoma (NK/TCL) were both of 4 cases, and the remaining one was peripheral T-cell lymphoma, unspecified (PTL, unspecified). Follow up data were available for 7 cases. Five patients including 2 with HSTCL, 2 with extranodal nasal type NK/TCL and one with PTL, unspecified died, with survival times ranged from 1 to 10 months. The other two patients are still alive, one with NK/TCL (two months+) and one with HSTCL (14+ months).

Conclusions: Splenic T-cell and NK-cell neoplasms are a group of uncommon lymphomas with heterogeneous clinicopathologic features and poor prognosis. A definite diagnosis must depend on clinical manifestations, histopathology, immunophenotype and TCR gene rearrangement analysis.