Escribano L, Orfao A, Díaz-Agustin B, Villarrubia J, Cerveró C, López A, Marcos M A, Bellas C, Fernández-Cañadas S, Cuevas M, Sánchez A, Velasco J L, Navarro J L, Miguel J F
Servicio de Hematología, Anatomía Patológica, Dermatología, and Inmunología, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain.
Blood. 1998 Apr 15;91(8):2731-6.
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 immunophenotypic 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.
本研究的目的是探讨骨髓肥大细胞（BMMC）免疫表型分析在惰性系统性肥大细胞病（SMCD）患者中的诊断价值。为此，共分析了10例SMCD患者和19例健康对照。我们的结果表明，SMCD患者的BMMC在光散射和免疫表型特征方面均与正常BMMC不同。因此，惰性SMCD患者的BMMC前向光散射（FSC）、侧向（90度）光散射（SSC）和基线自发荧光水平均高于对照受试者。从免疫表型角度来看，最显著的发现是SMCD患者的BMMC持续表达CD2（P = .0001）、CD25（P = .0001）和CD35（P = .06）分子，而所有正常对照均无这些标志物。相反，惰性SMCD患者的BMMC中不存在的CD71在正常受试者的BMMC中呈阳性。虽然在其他几个标志物中发现SMCD患者的BMMC与正常对照之间存在细微差异，但未达到统计学意义。总之，我们的结果表明，同时评估FSC/SSC以及CD117、CD2、CD25、CD33和CD35的反应性构成了成人SMCD患者BMMC免疫表型特征的基础，并且应该与临床和形态学研究相结合用于疾病的诊断。
