Serum tryptase measurements in patients with myelodysplastic syndromes.
Serum tryptase measurements in patients with myelodysplastic syndromes.
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骨髓增生异常综合征患者的血清类胰蛋白酶测量。
DOI：
10.1080/10428190290021470
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发表时间：
2002
影响因子：
2.6
通讯作者：
Valent,P
中科院分区：
文献类型：
--
作者：
Sperr,WR;Stehberger,B;Wimazal,F;Baghestanian,M;Schwartz,LB;Kundi,M;Semper,H;Jordan,JH;Chott,A;Drach,J;Jäger,U;Geissler,K;Greschniok,A;Horny,HP;Lechner,K;Valent,P
造血细胞的异常分化和成熟是骨髓增生异常综合征（MDS）的特征。类胰蛋白酶（α 型和 β 型）是谱系限制性丝氨酸蛋白酶，主要在肥大细胞 (MC) 中表达。我们分析了 89 名新生MDS 患者（难治性贫血 (RA)，n= 30；RA 伴环状铁粒幼细胞 (RARS)，n= 21；RA 伴原始细胞过多 (RAEB/RAEB-t)，n= 27；慢性粒单核细胞白血病 (CMML)，n= 11）中的类胰蛋白酶表达。通过 FIA 测量总类胰蛋白酶（α - 类胰蛋白酶 + β - 类胰蛋白酶）的血清水平。通过免疫组织化学和形态测定法测定石蜡包埋的骨髓（bm）切片中类胰蛋白酶+细胞的数量。在健康个体中，血清总类胰蛋白酶水平范围在 < 1 至 15 ng/ml (5.6 ± 2.8 ng/ml) 之间。在 5/22 例 RA 患者（22.7%）、4/17 例 RARS 患者（23.5%）、0/16 例 RAEB/RAEB-t 患者和 3/8 例 CMML 患者（37.5%）中检测到类胰蛋白酶水平 > 20 ng/ml。因此，与 RAEB/-t (8.7 ± 3.8) 相比，RA (16.6 ± 14.3 ng/ml)、RARS (12.9 ± 8.2) 和 CMML (16.5 ± 7.6) 的血清类胰蛋白酶浓度较高。通过形态测量，与对照组（54 ± 51 个细胞/mm2）相比，所有 MDS 组中均检测到类胰蛋白酶+bm 细胞数量增加（RA：139 ± 131；RARS：118 ± 98；RAEB/RAEB-t：80 ± 79；CMML：105 ± 114 个细胞/mm2）。通过 Northern 印迹和蛋白质分析评估，MDS 中的 bm 细胞主要产生 α-（原）类胰蛋白酶，但很少或不产生 β-类胰蛋白酶。总之，我们的数据表明，在一组 MDS 患者中可检测到类胰蛋白酶水平升高，这可能是因为产生这种酶的肿瘤（肥大）细胞增加。此外，血清类胰蛋白酶水平似乎与 MDS 变异相关。后续研究应阐明 MDS 中类胰蛋白酶浓度升高是否具有预后意义。
Abnormal differentiation and maturation of hemopoietic cells are characteristic features of myelodysplastic syndromes (MDS). Tryptases (α- and β-type) are lineage-restricted serine proteases primarily expressed in mast cells (MC). We have analyzed expression of tryptase in 89de novoMDS patients (refractory anemia (RA),n= 30; RA with ringed sideroblasts (RARS),n= 21; RA with excess of blasts (RAEB/RAEB-t),n= 27; chronic myelomonocytic leukemia (CMML),n= 11). Serum levels of total tryptase (α – protryptase + β – tryptase) were measured by FIA. The numbers of tryptase+cells were determined in paraffin-embedded bone marrow (bm) sections by immunohistochemistry and morphometry. In healthy individuals, serum total tryptase levels ranged between < 1 and 15 ng/ml (5.6 ± 2.8 ng/ml). Tryptase levels of > 20 ng/ml were detected in 5/22 patients with RA (22.7%), 4/17 with RARS (23.5%), 0/16 with RAEB/RAEB-t, and 3/8 with CMML (37.5%). Thus, serum tryptase concentrations were higher in RA (16.6 ± 14.3 ng/ml), RARS (12.9 ± 8.2), and CMML (16.5 ± 7.6) compared to RAEB/-t (8.7 ± 3.8). By morphometry, elevated numbers of tryptase+bm cells were detected in all MDS groups (RA: 139 ± 131; RARS: 118 ± 98; RAEB/RAEB-t: 80 ± 79; CMML: 105 ± 114 cells/mm2) compared to controls (54 ± 51 cells/mm2). As assessed by Northern blotting and protein analysis, bm cells in MDS primarily produced α-(pro)tryptase, but little or no β-tryptase. Together, our data show that elevated levels of tryptase are detectable in a group of patients with MDS probably because of an increase in neoplastic (mast) cells producing the enzyme(s). In addition, serum tryptase levels appear to correlate with MDS variants. Follow up studies should clarify whether an elevated tryptase concentration in MDS is of prognostic significance.