Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis
Tryptase detection in bone-marrow blood: A new diagnostic tool in systemic mastocytosis
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DOI：
10.1016/j.jaad.2006.09.020
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发表时间：
2007-03-01
影响因子：
13.8
通讯作者：
Bauer, Ralf
中科院分区：
文献类型：
--
作者：
Proelss, Julia;Wenzel, Joerg;Bauer, Ralf
背景：肥大细胞增多症包括一组以肥大细胞异常生长和积累为特征的异质性疾病。血清类胰蛋白酶（一种必需的肥大细胞酶）的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。 目的。我们假设检测骨髓血中的类胰蛋白酶可能会提供有关肥大细胞增多症患者骨髓受累的额外、更敏感的信息。方法：对有皮肤症状的患者 (n = 17)、有皮肤外症状的患者 (n = 16) 和健康对照受试者 (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）和对照受试者（n = 7）的骨髓活检标本进行组织学研究，并对这些个体的骨髓血中类胰蛋白酶水平进行分析。结果：我们首次检测到系统性肥大细胞增多症患者骨髓血中类胰蛋白酶水平显着升高。其次，我们可以提出血清类胰蛋白酶水平与肥大细胞增多症临床症状之间的明确相关性。 局限性：通过本研究，我们建立了一种新的系统性肥大细胞增多症诊断工具。不幸的是，我们只能提供有限的病例，因为系统性肥大细胞增多症是一种罕见疾病，涉及少数患者。结论：我们的结果表明，骨髓血中类胰蛋白酶的测量是系统性肥大细胞增多症患者骨髓中肥大细胞负荷的新的、敏感的标志物。
Background: 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.Objective. 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.Methods: 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.Results: 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.Limitations: 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.Conclusion: 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.