Indolent systemic mast cell disease in adults:: Immunophenotypic characterization of bone marrow mast cells and its diagnostic implications
Indolent systemic mast cell disease in adults:: Immunophenotypic characterization of bone marrow mast cells and its diagnostic implications
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DOI：
10.1182/blood.v91.8.2731.2731_2731_2736
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发表时间：
1998-04-15
期刊：
影响因子：
20.3
通讯作者：
San Miguel, JF
中科院分区：
文献类型：
--
作者：
Escribano, L;Orfao, A;San Miguel, JF
本研究的目的是探讨骨髓肥大细胞（BMMC）免疫表型分析对惰性系统性肥大细胞病（SMCD）患者的诊断价值。为此，总共对 10 名 SMCD 患者和 19 名健康对照进行了分析。我们的结果表明，来自 SMCD 的 BMMC 在光散射和免疫表型特征方面均不同于正常 BMMC。因此，惰性 SMCD 患者的 BMMC 中的前向光散射 (FSC)、侧向（90 度）光散射 (SSC) 和基线自发荧光水平高于对照受试者。从免疫表型的角度来看，最引人注目的发现是 SMCD 患者的 BMMC 持续表达 CD2 (P = .0001)、CD25 (P = .0001) 和 CD35 (P = .06) 分子，这些标记物在所有正常对照中均不存在。相比之下，惰性 SMCD 的 BMMC 中不存在 CD71，而正常受试者的 BMMC 中则呈阳性。尽管 SMCD 患者的 BMMC 与正常对照之间的其他几个标志物存在细微差异，但并未达到统计学显着性。总之，我们的结果表明，同时评估 FSC/SSC 和 CD117、CD2、CD25、CD33 和 CD35 的反应性构成了成人 SMCD BMMC 免疫表型特征的基础，并且应与疾病诊断的临床和形态学研究相结合。 (C) 1998 年，美国血液学会。
The aim of the present study was to explore the diagnostic value of the immunophenotypic analysis of bone marrow mast cells (BMMC) in indolent systemic mast cell disease (SMCD) patients. For that purpose, a total of 10 SMCD patients and 19 healthy controls were analyzed. Our results show that BMMC from SMCD are different from normal BMMC with regard to both their light scatter and immuno-phenotypic characteristics. Accordingly, forward light scatter (FSC), side (90 degrees) light scatter (SSC), and baseline autofluorescence levels were higher in BMMC from indolent SMCD patients than they were in control subjects. From the immunophenotypic point of view, the most striking findings were the constant expression of CD2 (P =.0001), CD25 (P =.0001), and CD35 (P =.06) molecules by BMMC from SMCD patients, markers that were absent from all normal controls. In contrast, CD71, absent in BMMC from indolent SMCD, was positive in BMMC from normal subjects. Although, slight differences between BMMC from SMCD patients and normal controls were found in several other markers, they did not reach statistical significance. In conclusion, our results show that simultaneous assessment of FSC/SSC and reactivity for the CD117, CD2, CD25, CD33, and CD35 forms the basis for the immunophenotypic characterization of BMMC from SMCD in adults and should be integrated with clinical and morphologic studies for the diagnosis of the disease. (C) 1998 by The American Society of Hematology.