Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine.

Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine.
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2-氯脱氧腺苷成功治疗白血病期肝脾 γ δ T 细胞淋巴瘤。

DOI:
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发表时间:
2002
期刊:
Haematologia
影响因子:
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通讯作者:
K. Pálóczi
K. Pálóczi
中科院分区:
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文献类型:
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作者:
L. Gopcsa;A. Bányai;J. Tamáska;A. Karádi;A. Matolcsy;K. Pálóczi

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本文报告一位39岁女性罹患肝脾γ δ T细胞淋巴瘤合并白血病的罕见病例。在第一次介绍,她有脾肿大和全血细胞减少诊断为脾功能亢进治疗脾切除术。随后,她出现肝肿大和进行性肿瘤性淋巴细胞增多症。骨髓显示中等大小细胞的窦状浸润。外周血单个核细胞的流式细胞术分析表明表达CD 3、CD 7、CD 16、CD 56抗原和T细胞受体γ δ。聚合酶链反应检测单克隆TCR γ和β链基因重排。患者接受传统化疗和α-干扰素治疗,但未成功。因此,引入2-氯脱氧腺苷导致6个月的完全缓解。报告的情况下,证明了有用的2-氯脱氧腺苷治疗肝脾γ δ T细胞淋巴瘤。
An unusual case of hepatosplenic gamma delta T-cell lymphoma with leukemic phase in a 39-year-old woman is reported. At the first presentation she had splenomegaly and pancytopenia diagnosed as hypersplenia treated by splenectomy. Subsequently, she developed hepatomegaly and progressive neoplastic lymphocytosis. The bone marrow showed a sinusoidal infiltrate of medium-sized cells. Flowcytometric analysis of peripheral blood mononuclear cells demonstrated expression of CD3, CD7, CD16, CD56 antigens and T-cell receptor gamma delta. A monoclonal TCR gamma- and beta-chain gene rearrangement were detected by polymerase chain reaction. The patient was treated by traditional chemotherapy and alpha-interferon, unsuccessfully. Therefore, 2-chlorodeoxyadenosine was introduced resulting in a complete remission for 6 months. The reported case demonstrates the usefulness of 2-chlorodeoxyadenosine in treatment of hepatosplenic gamma delta T-cell lymphoma.