@article{oai:ir.kagoshima-u.ac.jp:00013033,
 author = {YAMAMOTO, Noriko and FUJISAKI, Hitomi and UEMURA, Yoshiko and TOKUNAGA, Masayoshi and KUBOTA, Ryuji and TARA, Mitsutoshi and NIINA, Kiyoshige and MIZUTAMARI, Koukichi},
 issue = {Suppl. 2},
 journal = {鹿児島大学医学雑誌=Medical journal of Kagoshima University},
 month = {2016-10-31},
 note = {A Trial of Application of the Updated Kiel Classification to Extranodal Malignant Lymphomas with Advice of K. Lennert and AC Feller : Proceedings of the Second Seminar on Malignant Lymphoma in Kagoshima, Japan. April 13 - 15, 1994, We described a case of splenic CD4 positive γ/δ T-cell lymphoma with bone marrow involvement, and leukemic change in the terminal stage. A 23-year-old male had high fever and hepatosplenomegaly with pancytopenia and histiocytic hemophagocytosis. In order to make an accurate diagnosis and to improve symptom, splenectomy was performed. Histological section of the spleen showed marked congestion and diffuse proliferation of atypical lymphocytes having oval nuclei with or without nuclear indentation in the red pulp, indicating diffuse, large cell lymphoma. Immunohistochemically, the atypical cells were positive for CD2, CD3, CD4, CD15 and CD25. The lymphoma cells were also positive for δTCS1 and negative for TCR βF1. Despite the chemotherapy, his lymphoma did not improve and developed into leukemic change in the critical state. When his bone marrow was largely involved by blastic cells about 90%, phenotype examination by flow cytometry revealed CD3-positive and TCR γ δ -1-positive, but CD 4-negative. This case is thought to be an unusual splenic γ/δ T-cell
lymphoma with onset of histiocytic hemophagocytosis and CD4 expression in the initial stage. There are two forms of the T-cell receptor ; TCR α/β and TCR γ/δ. The former is expressed on the majority of peripheral T-cells, whereas the latter is present on the minority, reported about 9% of the peripheral blood lymphocytes^{1)}. These TCR γ/δ positive lymphocytes in the peripheral blood are small to medium-sized cells in normal donors. The spleen is the organ with highest population of TCR γ/δ lymphocytes with preferential location in the sinusoids of red pulp while TCR α/β lymphocytes occupies the periarteriolar sheaths of penicillary arteries. The marker expression of peripheral γ/δ lymphocytes are usually double negative, but there are only a few single positive lymphocytes for CD4 or CD8. A similar distribution has been demonstrated in patients with T-cell lymphoma (T-ML); i.e., most peripheral T-ML cells express TCR α/β, whereas TCR γ/δ expression is very rare. Since the first report by Farcet et al^{2)} on 1990, only a few more than ten cases with γ/δ T-ML have been reported. The majority of these cases shows hepatosplenic and bone marrow involvement with sinusal/sinusoidal pattern by infiltration of mdeium sized abnormal lymphocytes without azurophilic granules, male predominance, marker expression of CD3, δTCS1, TCRδ1, but negative for CD4,5,8, and TCRβF1^{2-6)}. These tumor cells were also characterized by the genotypic expression of monoclonal rearrangement of TCR γ and δ. We describe an unusual case of T-ML with TCR γ/δ expression arising in the spleen.},
 pages = {141--145},
 title = {Splenic gamma/delta T-cell Lymphoma Report of a Case},
 volume = {47},
 year = {}
}