Itoh K, Inaba T, Murakami S, Oku N, Takeda N, Ura Y, Shimazaki C, Nakanishi S, Nakagawa M, Taniwaki M
Second Department of Internal Medicine, Kyoto Prefectural University of Medicine.
Rinsho Ketsueki. 1990 Oct;31(10):1721-5.
A 32-year-old female was admitted to our hospital because of abdominal fullness, jaundice and pretibial edema in November, 1987. Leukocyte count of peripheral blood showed 13300/microliters with 84% leukemic cells and bone marrow was normocellular with 63.6% leukemic cells. Leukemic cells had azurophilic large granules with basophilic cytoplasm and positive for CD 2, OKIa 1, NKH-1 and negative for CD 3, CD 4, CD 8, CD 16. T cell receptor (TCR) genes for beta, gamma and delta chain and immunoglobulin heavy chain gene were in germ line configuration. These cells also had natural killer (NK) activity and antibody dependent cell mediated cytotoxicity (ADCC) activity. These observations suggested that they were derived from NK cell lineage. It is often difficult to demonstrate their clonalities in lymphoproliferative disorder of granular lymphocytes (LDGL). In the present case, the analysis of EBV genome using termini probe demonstrated polyclonal bands, while we found constant chromosome abnormalities; 47 XX, +3. From these observations, this case was considered to have several clones, and one of which could be detected by chromosome analysis. The analysis of EBV genome using termini probe may be useful to demonstrate their clonalities in LDGL in addition to conventional chromosome analysis.
1987年11月，一名32岁女性因腹胀、黄疸和胫前水肿入院。外周血白细胞计数显示为13300/微升，其中白血病细胞占84%，骨髓细胞正常，白血病细胞占63.6%。白血病细胞有嗜天青大颗粒，胞质嗜碱性，CD 2、OKIa 1、NKH-1呈阳性，CD 3、CD 4、CD 8、CD 16呈阴性。β、γ和δ链的T细胞受体（TCR）基因以及免疫球蛋白重链基因呈种系构型。这些细胞还具有自然杀伤（NK）活性和抗体依赖性细胞介导的细胞毒性（ADCC）活性。这些观察结果表明它们源自NK细胞系。在颗粒淋巴细胞增殖性疾病（LDGL）中，通常很难证明它们的克隆性。在本病例中，使用末端探针分析EBV基因组显示多克隆条带，同时我们发现了恒定的染色体异常；47 XX，+3。根据这些观察结果，该病例被认为有几个克隆，其中一个可以通过染色体分析检测到。除了传统的染色体分析外，使用末端探针分析EBV基因组可能有助于证明LDGL中的克隆性。
