Nodal gamma/delta T cell lymphoma in complete remission following allogeneic bone marrow transplantation from an HLA-matched unrelated donor.
Gamma/delta T cell lymphoma is very rare, and usually occurs as an extranodal tumor. We describe the case of a 16-year-old Japanese man with an unusual nodal gamma/delta T cell lymphoma with generalized lymphadenopathy and bone marrow involvement. No tumor involvement was observed in the liver, spleen, or nasal cavity. Examination for surface antigens on lymphoma cells revealed a unique phenotype, positive for CD3 and T cell receptor (TCR) gamma/delta, but negative for CD2. Genotypic analysis revealed the tumor to be of monoclonal origin and characterized by TCR gamma-chain gene rearrangement, but there was no rearrangement of the TCR beta-chain gene. Our patient's tumor responded to combination chemotherapy and subsequent allogeneic bone marrow transplantation from an HLA-matched unrelated donor. He has remained well and free of disease for 35 months.
Aoyama
Y
Y
Department of Clinical Hematology, Osaka City University Medical School, 1-4-3, Asahimachi, Abeno-ku, Osaka 545-8586, Japan.
Yamane
T
T
Hino
M
M
Ota
K
K
Hasegawa
T
T
Sakamoto
C
C
Nakamae
H
H
Yamamura
R
R
Koh
K R
KR
Takubo
T
T
Inoue
T
T
Tsubaki
K
K
Tatsumi
N
N
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016399:Lymphoma, T-Cell / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D012074:Remission Induction; D014184:Transplantation, Homologous; D014750:Vincristine / Q000008:administration & dosage
D016692:Receptors, Antigen, T-Cell, gamma-delta; D003561:Cytarabine; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; D001215:Asparaginase