Gamma/delta T cell lymphoma.
A 54-year-old woman complained of fever and hepato-splenomegaly. The pathological findings of a liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids and that of the laparoscopically resected spleen revealed the infiltration of lymphocytes in the red pulp, which was positive for CD3, CD43, CD45RO and T-cell intracellular antigen-1 (TIA-1) and was negative for betaF1, while the white pulp was spared. Genetic analysis of the spleen cells revealed the rearrangement of T-cell receptor (TCR) Cbeta1, Jdelta1 and Jgamma. Epstein-Barr virus (EBV) genomic DNA was detected in the spleen cells. Atypical lymphocytes appeared in the peripheral blood and bone marrow, chromosomal analysis revealed del (13) (q12 q14), trisomy 8 and breakage of RB gene. Elevated level of serum vascular endothelial growth factor (VEGF) was observed. Hepatosplenic gammadelta T cell lymphoma (GDTL) was diagnosed. The patient was treated with chemotherapy by cyclophosphamide, hydroxydoxorubicin, vincristine and prednisolone (CHOP), however, it was ineffective, and the patient died of hemorrhage from the lymphoma involvement of the intestine 5 months after the onset of disease.
Division of Hematology, Department of Medicine, Yamaguchi Prefecture Central Hospital, Hofu 747-8511.
Akihiko
A
Taguchi
Mutsuko
M
Miyazaki
Shizu
S
Sakuragi
Kenji
K
Shinohara
Toshiaki
T
Kamei
Yusuke
Y
Inoue
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000502:physiology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004279:DNA, Viral / Q000032:analysis; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005434:Flow Cytometry; D015329:Gene Rearrangement, T-Lymphocyte; D006529:Hepatomegaly; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008113:Liver Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D013154:Spleen / Q000378:metabolism / Q000473:pathology; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D013163:Splenomegaly; D042461:Vascular Endothelial Growth Factor A / Q000097:blood; D014750:Vincristine / Q000627:therapeutic use
D004279:DNA, Viral; D016692:Receptors, Antigen, T-Cell, gamma-delta; D042461:Vascular Endothelial Growth Factor A; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone