Cytogenetic, cytophotometric, and ultrastructural study of large cerebriform cells of the Sézary syndrome and description of a small-cell variant.
Cytogenetic, cytophotometric, and ultrastructural study of large cerebriform cells of the Sézary syndrome and description of a small-cell variant.
复制标题
对塞扎里综合征大脑状细胞的细胞遗传学、细胞光度测量和超微结构研究以及小细胞变异的描述。
DOI：
10.1093/jnci/50.5.1145
复制
发表时间：
1973
期刊：
影响因子：
--
通讯作者：
M. Prunieras
中科院分区：
文献类型：
--
作者：
M. Lutzner;I. Emerit;R. Durepaire;G. Flandrin;C. Grupper;M. Prunieras
对4例Sézary综合征患者进行了超微结构、细胞光度和细胞遗传学研究。Sézary综合征的诊断是基于皮肤表现与血液中存在异常细胞的相关性，异常细胞表现为高核质比、有凹槽和折叠的核、过氧化物酶和非特异性酯酶阴性。12例患者作为对照：1例单核细胞白血病，6例慢性淋巴细胞白血病（CLL），2例健康供体，3例其他皮肤病患者。识别出两种类型的异常细胞：1）具有蛇形和脑形核、近四倍体DNA值和近四倍体染色体计数的大细胞类型和2）具有缩进核、二倍体DNA值和假二倍体或超二倍体染色体计数的小细胞变体。除其他外，还发现了A和B以及Gq-标记。得出的结论是，染色体异常代表了表征Sézary细胞的另一个标准，其性质似乎是淋巴细胞，因为其在培养中对植物血凝素的反应。此外，初步的免疫学研究表明，Sézary细胞不是B淋巴细胞。假设Sézary综合征是一种不常见的胸腺源性CLL。
Ultrastrudural, cytophotometric, and cytogenetic studies were performed on 4 patients with Sézary syndrome. The diagnosis of Sézary syndrome was based on the association of skin manifestations with the presence in the blood of an abnormal cell exhibiting a high nucleocytoplasmic ratio, a grooved and folded nucleus, and peroxidase and nonspecific esterase negativity. Twelve patients were used as controls: 1 with monocytic leukemia, 6 with chronic lymphocytic leukemia (CLL), 2 healthy donors, and 3 patients with other skin diseases. Two types of abnormal cells were recognized: 1) a large-cell type with a serpentine and cerebriform nucleus, near-tetraploid DNA values, and near-tetraploid chromosome counts and 2) a small-cell variant with indented nucleus, diploid DNA values,and pseudodiploid or hyperdiploid chromosome counts. Among others, A and B as well as Gq— markers were found. It was concluded that chromosomal abnormalities represent an additional criterion for the characterization of the Sézary cell, the nature of which appears to be lymphocytic because of itsresponse in culture to phytohemagglutinin. In addition, preliminary immunologic studies indicated that the Sézary cell is not a B-lymphocyte. It was hypothesized that Sézary syndrome is an uncommon thymus-derived form of CLL.