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Title   A Case of a Young Woman with Hepatosplenic γδ T - cell Lymphoma
Publicationinfo   2001 Jan; 033(03): 264-269.
Key_word   Hepatosplenic lymphoma , γδ T - cell receptor ( TCR ) , Leukemic phase , Young woman
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Abstract   Most T - cell lymphomas arise from mature αβ T - cells and commonly involve the nodes. Lymphomas bearing the γδ T - cell receptor (TCR) are very rare, and involve the lymph nodes minimally, if at all. He patosplenic γδ T - cell lymphoma is a recently identified , rare entity in which lymphoma cells bearing the γδ TCR infiltrate the sinusoids of the liver, splenic red pulp, and bone marrow. Its leukemic transformation is even more rare. Recently, we experienced a case of hepatosplenic γδ T - cell lymphoma in a 19-year-old woman who presented with epigastric pain, fever, massive splenomegaly, and pancytopenia. The splenectomy specimen and excisional biopsy of the liver revealed the infiltration of atypical T lymphocytes with the immunophenotypic markers of CD3 (+), CD45RO (pan-T antigen) (+), TIA-1 (+), CD4 (-). CD8 (-), CD56 (-), and s100 (-) in the sinusoids of the liver and splenic red pulp. Polymerase chain reaction (PCR) showed that these cells had the expression of the TCR γ gene rearrangements. Though the pancytopenia had improved after the splenectomy, the response of chemotherapy was transient. Her disease progressed rapidly and she expired in the leukemic phase. We report a case of hepatosplenic γδ T - cell lymphoma that developed in a young woman, along with a brief review of the literature. (Cancer Res earch and Treatment 2001; 33:264 - 268)
저 자   박일권(Il Gwon Park),서철원(Cheol Won Suh),허주령(Joo Ryung Huh),김선종(Sun Jong Kim),박건욱(Keon Uk Park),박성제(Seong Je Park),서돈대(Don Dae Seo),안만수(Man Su Ahn),장근두(Guun Doo Jang),김우건(Woo Kun Kim)