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B细胞疾病的免疫特征及慢性淋巴细胞白血病诊断评分系统的提议。

The immunological profile of B-cell disorders and proposal of a scoring system for the diagnosis of CLL.

作者信息

Matutes E, Owusu-Ankomah K, Morilla R, Garcia Marco J, Houlihan A, Que T H, Catovsky D

机构信息

Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.

出版信息

Leukemia. 1994 Oct;8(10):1640-5.

PMID:7523797
Abstract

We have investigated the role of immunophenotyping in distinguishing between leukemic B-cell lymphoproliferative disorders. Circulating cells from 666 cases were analyzed with a panel of markers by flow cytometry. The diseases included: chronic lymphocytic leukemia (CLL), 400; prolymphocytic leukemia, 22; hairy cell leukemia (HCL), 40; HCL variant, 15; splenic lymphoma with villous lymphocytes, 100; follicular lymphoma, 26; lymphoplasmacytic lymphoma, 25; mantle-cell lymphoma, 20; and large cell lymphoma, 18. On the basis of the most common marker profile in CLL, CD5+, CD23+, FMC7- and weak expression (+/-) of surface immunoglobulin (SmIg) and CD22, we devised a scoring system that gives for each of these five markers a value of 1 or 0 according to whether it is typical or atypical for CLL. Scores range from 5 (typical of CLL) to 0 (atypical for CLL). Application of the scoring system to all the cases showed that 87% of CLL scored 5 and 4 and only 0.4% scored 0 or 1, whereas 89% of other B-cell leukemias and 72% of lymphomas scored 0 or 1; only one case (0.3%) scored 4 and none scored 5 (p < 0.0001). There were no differences between CLL with high and low scores but higher scores were found in cases with more typical morphology (p < 0.0015). Considering each individual marker, there was no single one that distinguished CLL from other diseases, although the most reliable were SmIg intensity and FMC7. The proposed score will facilitate the diagnosis of B-lymphoproliferative disorders and improve their classification.

摘要

我们研究了免疫表型分析在区分白血病性B细胞淋巴增殖性疾病中的作用。采用流式细胞术,用一组标志物对666例患者的循环细胞进行了分析。这些疾病包括:慢性淋巴细胞白血病(CLL)400例;幼淋巴细胞白血病22例;毛细胞白血病(HCL)40例;HCL变异型15例;伴绒毛淋巴细胞的脾淋巴瘤100例;滤泡性淋巴瘤26例;淋巴浆细胞淋巴瘤25例;套细胞淋巴瘤20例;以及大细胞淋巴瘤18例。基于CLL中最常见的标志物谱,即CD5 +、CD23 +、FMC7 -以及表面免疫球蛋白(SmIg)和CD22的弱表达(+/-),我们设计了一种评分系统,根据这五个标志物中的每一个对于CLL来说是典型还是非典型,分别给予1或0分。分数范围从5分(典型的CLL)到0分(非典型的CLL)。将该评分系统应用于所有病例显示,87%的CLL得分为5分和4分,只有0.4%得分为0分或1分,而89%的其他B细胞白血病和72%的淋巴瘤得分为0分或1分;只有1例(0.3%)得分为4分,没有病例得5分(p < 0.0001)。高分和低分的CLL之间没有差异,但在形态更典型的病例中发现分数更高(p < 0.0015)。考虑每个单独的标志物,没有一个标志物能将CLL与其他疾病区分开来,尽管最可靠的是SmIg强度和FMC7。所提出的评分将有助于B淋巴增殖性疾病的诊断并改善其分类。

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