Proelss Julia, Wenzel Joerg, Ko Yon, Bieber Thomas, Bauer Ralf
Department of Dermatology, University of Bonn, Bonn, Germany.
J Am Acad Dermatol. 2007 Mar;56(3):453-7. doi: 10.1016/j.jaad.2006.09.020. Epub 2006 Oct 30.
The condition mastocytosis includes a heterogenous group of disorders that are characterized by abnormal growth and accumulation of mast cells. The detection of serum tryptase, an essential mast cell enzyme, is a widely used tool in the diagnosis of mastocytosis. The diagnosis of systemic mastocytosis is substantially based on the histologic examination of bone-marrow biopsy specimens.
We hypothesized that the detection of tryptase in bone-marrow blood might provide additional, more sensitive information on the bone-marrow involvement of patients with mastocytosis.
Serum tryptase was monitored in patients with cutaneous symptoms (n = 17), patients with extracutaneous symptoms (n = 16), and healthy control subjects (n = 359). Bone-marrow biopsy specimens of patients with systemic mastocytosis (n = 7) and control subjects (n = 7) were investigated histologically and bone-marrow blood of these individuals was analyzed on the tryptase levels.
We could detect for the first time significantly elevated tryptase levels in bone-marrow blood of patients with systemic mastocytosis. Secondarily we could present a clear correlation between the level of serum tryptase and the clinical symptoms of mastocytosis.
With the present study, we establish a new diagnostic tool for systemic mastocytosis. Unfortunately, we can only present a limited number of cases, since systemic mastocytosis is a rare disease involving few patients.
Our results indicate that the measurement of tryptase in bone-marrow blood is a new, sensitive marker of the mast cell burden in bone marrow of patients with systemic mastocytosis.
肥大细胞增多症包括一组异质性疾病,其特征为肥大细胞异常生长和积聚。血清类胰蛋白酶(一种重要的肥大细胞酶)检测是肥大细胞增多症诊断中广泛使用的工具。系统性肥大细胞增多症的诊断主要基于骨髓活检标本的组织学检查。
我们推测检测骨髓血中的类胰蛋白酶可能为肥大细胞增多症患者的骨髓受累情况提供更多、更敏感的信息。
对有皮肤症状的患者(n = 17)、有皮肤外症状的患者(n = 16)和健康对照者(n = 359)的血清类胰蛋白酶进行监测。对系统性肥大细胞增多症患者(n = 7)和对照者(n = 7)的骨髓活检标本进行组织学研究,并分析这些个体骨髓血中的类胰蛋白酶水平。
我们首次检测到系统性肥大细胞增多症患者骨髓血中的类胰蛋白酶水平显著升高。其次,我们发现血清类胰蛋白酶水平与肥大细胞增多症的临床症状之间存在明显相关性。
通过本研究,我们建立了一种用于系统性肥大细胞增多症的新诊断工具。遗憾的是,由于系统性肥大细胞增多症是一种罕见疾病,患者数量较少,我们只能展示有限数量的病例。
我们的结果表明,检测骨髓血中的类胰蛋白酶是系统性肥大细胞增多症患者骨髓中肥大细胞负荷的一种新的敏感标志物。