Roberts L J, Oates J A
Department of Pharmacology, Vanderbilt University, Nashville, Tennessee 37232-6602, USA.
J Invest Dermatol. 1991 Mar;96(3 Suppl):19S-24S; discussion 24S-25S; 60S-65S. doi: 10.1111/1523-1747.ep12468945.
Systemic mastocytosis is characterized by an abnormal proliferation of tissue mast cells. Symptoms of mastocytosis are primarily attributed to the release of mast cell mediators during episodes of systemic activation of the excessive numbers of mast cells. Thus, biochemical evidence for the release of increased quantities of mast cell secretory products can suggest or confirm, depending on the clinical situation, a diagnosis of systemic mastocytosis. A major advantage of the biochemical approach to the diagnosis of systemic mast cell disease is that it has allowed the recognition of a class of patients in whom episodes of systemic mastocyte activation can be unequivocally documented biochemically but in whom clear-cut evidence of abnormal mast cell proliferation is lacking by current histologic criteria. Although the release of increased quantities of mast cell mediators can be demonstrated during episodes of mast cell activation in such patients, mediator levels are usually normal at quiescent times. By contrast, patients with proliferative mast cell disease (mastocytosis) usually exhibit chronic overproduction of mast cell mediators. Mast cell secretory products that can be measured in an attempt to obtain biochemical evidence of systemic mast cell activation include histamine, prostaglandin D2, tryptase, and heparin. The analytical approaches to assessing release of those individual mast cell products are evaluated. In general, the diagnosis and investigation of patients with systemic mast cell activation can best be accomplished by concerted use of histologic examination of key tissues together with analysis of chemical markers of the mast cell.
系统性肥大细胞增多症的特征是组织肥大细胞异常增殖。肥大细胞增多症的症状主要归因于在大量肥大细胞全身激活发作期间肥大细胞介质的释放。因此，根据临床情况，肥大细胞分泌产物数量增加的生化证据可提示或确诊系统性肥大细胞增多症。生化方法诊断系统性肥大细胞疾病的一个主要优点是，它能够识别出一类患者，在这类患者中，可通过生化方法明确记录全身肥大细胞激活发作，但根据目前的组织学标准缺乏明确的肥大细胞异常增殖证据。尽管在此类患者的肥大细胞激活发作期间可证明肥大细胞介质释放量增加，但在静止期介质水平通常正常。相比之下，增殖性肥大细胞疾病（肥大细胞增多症）患者通常表现出肥大细胞介质的慢性过度产生。为了获得系统性肥大细胞激活的生化证据而可测量的肥大细胞分泌产物包括组胺、前列腺素D2、类胰蛋白酶和肝素。对评估这些单个肥大细胞产物释放的分析方法进行了评估。一般来说，系统性肥大细胞激活患者的诊断和研究最好通过联合使用关键组织的组织学检查以及肥大细胞化学标志物分析来完成。
