Hepatosplenic gammadelta T-cell lymphoma: relation to Epstein-Barr virus and activated cytotoxic molecules.
OBJECTIVE
Hepatosplenic gammadelta T-cell lymphoma (TCL) is a rare, aggressive subset of peripheral TCL that presents with hepatosplenomegaly and cytopenia. Epstein-Barr virus (EBV) infection and activated cytotoxic molecules (granzyme and perforin) are uncommon in hepatosplenic gammadelta CTL. EBV infection and activated cytotoxic molecules are occasionally detected in non-hepatosplenic gammadelta TCL. We describe the clinicopathological features of three Japanese cases who were not immunodeficient.


RESULTS
All cases showed gammadelta T-cell type (CD2+, CD3+, T-cell receptor (TCR)delta-1+, betaF1-). Two cases expressed natural killer (NK) cell-associated antigens (CD8-, CD16+, CD56+; CD8-, CD16-, CD56+), and one expressed CD8 (CD8+, CD16-, CD56-). All cases expressed cytotoxicity-associated molecules (perforin, granzyme B, TIA-1 and Fas ligand). However, perforin and Fas ligand were not detected in one case. In-situ hybridization analysis with EBER probes revealed strong nuclear positivity in all neoplastic cells. In addition, two cases showed clonal bands of the EBV terminal repeat (TR) gene. Cytologically, instead of the presence of monomorphic medium-sized cells, our three cases showed pleomorphic medium-sized and large cells.


CONCLUSIONS
Our gammadelta TCL cases were clinicopathologically considered to be compatible with hepatosplenic gammadelta T-cell lymphoma. However, with regard to EBV association, activated cytotoxic profile and cytological features they resembled non-hepatosplenic gammadelta TCL. EBV may play a role in this disease by inducing cellular activation.
Ohshima
K
K
Department of Pathology, School of Medicine, Fukuoka University, Kyushu University, Fukuoka, Japan.
Haraoka
S
S
Harada
N
N
Kamimura
T
T
Suzumiya
J
J
Kanda
M
M
Kawasaki
C
C
Sugihara
M
M
Kikuchi
M
M
D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D053222:Fas Ligand Protein; D053804:Granzymes; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology* / Q000821:virology; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D008565:Membrane Proteins / Q000032:analysis; D054353:Perforin; D039102:Poly(A)-Binding Proteins; D052899:Pore Forming Cytotoxic Proteins; D011506:Proteins; D012367:RNA, Viral / Q000235:genetics; D016601:RNA-Binding Proteins / Q000032:analysis; D016692:Receptors, Antigen, T-Cell, gamma-delta; D012697:Serine Endopeptidases / Q000032:analysis; D013160:Splenic Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D000074019:T-Cell Intracellular Antigen-1; D013602:T-Lymphocytes, Cytotoxic / Q000378:metabolism
D015703:Antigens, CD; C505661:FASLG protein, human; D053222:Fas Ligand Protein; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D039102:Poly(A)-Binding Proteins; D052899:Pore Forming Cytotoxic Proteins; D011506:Proteins; D012367:RNA, Viral; D016601:RNA-Binding Proteins; D016692:Receptors, Antigen, T-Cell, gamma-delta; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D054353:Perforin; C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases