Successful treatment of Epstein-Barr virus-associated natural killer cell large granular lymphocytic leukaemia using allogeneic peripheral blood stem cell transplantation.
We report a case of natural killer cell large granular lymphocytic (NK-LGL) leukaemia successfully treated with allogeneic peripheral blood stem cell transplantation (allo-PBSCT). The peripheral blood (PB) revealed an abnormal expansion of LGL that were CD3-, CD16- and CD56+, and had natural killer activity. In situ EBER-1 hybridization of the PB mononuclear cells showed the presence of Epstein-Barr virus (EBV) in the LGL as well as in lymphocytes infiltrating the tonsils and colon. Southern blotting with an EBV-terminal repetitive sequence probe demonstrated clonal proliferation of EBV+ cells. The patient received allo-PBSCT from his HLA-matched sister with a conditioning regimen involving the use of cyclophosphamide and fractionated total body irradiation. The patient promptly recovered trilineage haematopoiesis without graft-versus-host disease, and has been in complete remission without therapy for 10 months since allo-PBSCT, suggesting that allo-PBSCT could eradicate the NK-LGL leukaemic cells.
Third Department of of Medicine, Kanazawa University School of Medicine, Ishikawa, Japan.
A
A
Takami
S
S
Nakao
A
A
Yachie
Y
Y
Kasahara
H
H
Okumura
Y
Y
Miura
N
N
Sugimori
W
W
Zeng
H
H
Wang
T
T
Kotani
S
S
Shiobara
T
T
Matsuda
D000293:Adolescent; D018380:Hematopoietic Stem Cell Transplantation; D004854:Herpesvirus 4, Human; D006801:Humans; D007694:Killer Cells, Natural; D015458:Leukemia, T-Cell / Q000628:therapy*; D008297:Male; D014184:Transplantation, Homologous