Smith J L, Oscier D G, Haegert D G, Jones D B, Howell M, Hodges E, Stevenson F K, Hamblin T J
Regional Immunology Service, Tenovus Research Laboratory, Southampton.
J Clin Pathol. 1988 Jul;41(7):746-52. doi: 10.1136/jcp.41.7.746.
A patient with CD3, CD8 positive lymphocytosis presented with features consistent with T cell chronic lymphocytic leukaemia/proliferations of large granular lymphocytes. The marrow and blood lymphoid populations (19.4 x 10(9)/l) contained more than 80% CD3 and CD8 positive cells with no evidence of a monotypic B cell population. A biopsy specimen of a vasculitic rash showed a diffuse infiltrate of CD3, CD8 positive cells into the upper dermis, consistent with T cell lymphocytic disease. After follow up for two years without treatment the blood lymphocyte count was 53 x 10(9)/l and was composed of cytologically small lymphocytes. A monoclonal SIg M D k lymphoid population (more than 90%) was demonstrable in sample blood and marrow aspirate. Gene rearrangement studies carried out on DNA extracted from peripheral blood lymphocytes at presentation and at two year follow up exhibited JH and Ck immunoglobulin gene rearrangement but no rearrangement of T cell receptor TcR gamma and beta genes. It is thought that this is the first well documented case of an aggressive CD8 positive lymphocytosis preceding, or in response to, an underlying B cell neoplasm.
一名CD3、CD8阳性淋巴细胞增多症患者表现出与T细胞慢性淋巴细胞白血病/大颗粒淋巴细胞增殖相符的特征。骨髓和血液中的淋巴细胞群体(19.4×10⁹/L)中,超过80%为CD3和CD8阳性细胞,未发现单克隆B细胞群体的证据。一份血管炎性皮疹的活检标本显示,CD3、CD8阳性细胞弥漫浸润至真皮上层,符合T细胞淋巴细胞疾病。未经治疗随访两年后,血液淋巴细胞计数为53×10⁹/L,由细胞学上的小淋巴细胞组成。在样本血液和骨髓穿刺物中可检测到单克隆SIg M D k淋巴细胞群体(超过90%)。对初诊时及随访两年时从外周血淋巴细胞中提取的DNA进行的基因重排研究显示,存在JH和Ck免疫球蛋白基因重排,但未发现T细胞受体TcRγ和β基因重排。据认为,这是首例有充分记录的在潜在B细胞肿瘤之前或对其产生反应的侵袭性CD8阳性淋巴细胞增多症病例。