Evolution of a terminal deoxynucleotidyl transferase-positive lymphoma from a chronic T cell lymphocytosis.

D G Oscier, G J Mufti, T J Hamblin, D B Jones, J L Smith
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引用次数: 4

Abstract

A 56-yr-old Caucasian man presented with a generalised scaly rash and a peripheral blood lymphocytosis of 5.6 X 10(9)/l. 5 yr later he developed cutaneous nodules, lymphadenopathy and hepatosplenomegaly. Cells with convoluted nuclei and prominent nucleoli were seen in the peripheral blood. He underwent splenectomy and received intensive chemotherapy but died 6 months later with CNS infiltration. At presentation the peripheral blood lymphocytes were E-ve, UCHT1 + ve, and OKT8 + ve. Following transformation, cells in blood, spleen and CSF were E-ve, OKT11 + ve, DR + ve and Tdt + ve. A proportion of these cells had a Sézary-like appearance at E/M. The splenic cells showed functional suppressor activity. This is the first reported case of the evolution of a Tdt + ve lymphoma from a post-thymic T cell lymphocytosis.

慢性T细胞淋巴细胞增多症的终末脱氧核苷酸转移酶阳性淋巴瘤的演变。
56岁白人男性,表现为全身性鳞状皮疹,外周血淋巴细胞增多5.6 X 10(9)/l。5年后出现皮肤结节、淋巴结病变和肝脾肿大。外周血中可见细胞核卷曲、核仁突出的细胞。他接受了脾切除术和强化化疗,但6个月后死于中枢神经系统浸润。发病时外周血淋巴细胞为E-ve、UCHT1 + ve、OKT8 + ve。转化后血、脾、CSF细胞E-ve、OKT11 + ve、DR + ve、Tdt + ve。这些细胞中有一部分在电镜下呈ssamzar样外观。脾细胞表现出功能性抑制活性。这是首次报道的由胸腺后T细胞淋巴细胞增多症演变为Tdt + ve淋巴瘤的病例。
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