某些实验室异常与系统性肥大细胞增多症(SM)的亚变异相关,并且通常与预后相关。为了评估SM中个体血清化学参数的诊断和预后价值,分析了在欧洲能力网络(ECNM)中招募的2607名患者和在德国嗜酸性粒细胞和肥大细胞注册(GREM)中招募的575名患者。对于SM的筛查和诊断,胰蛋白酶被确定为最特异的血清参数。为了区分惰性和高级SM(AdvSM),以下血清参数最相关:类胰蛋白酶,碱性磷酸酶(AP),β2-微球蛋白,乳酸脱氢酶(LDH),白蛋白,维生素B12和C反应蛋白(P<0.001)。关于AdvSM的子变体,LDH升高≥260U/L与多谱系扩增相关(白细胞增多,r=0.37,P<0.001;单核细胞增多,r=0.26,P<0.001)和相关髓样肿瘤的存在(P<0.001),而肥大细胞白血病的类胰蛋白酶水平最高(MCL与非MCL,308µg/Lvs.146微克/升,P=0.003)。基于多变量分析,乳酸脱氢酶1点的危险-风险加权分配(HR2.1[95%CI1.1-4.0],P=0.018)和β2-微球蛋白各1.5点(HR2.7[95%CI1.4-5.4],P=0.004)和白蛋白(HR3.3[95%CI1.7-6.5],P=0.001)描绘了一个高度预测性的三层风险分类系统(0分,8.1年vs.1分,2.5年,≥1.5分,1.7年;P<0.001)。此外,血清化学参数能够进一步对IPSM-AdvSM1/2风险评分分类患者进行分层(P=0.027).总之,血清化学谱分析是临床实践中支持SM及其亚变体诊断和预测的重要工具。
UNASSIGNED: Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most specific serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, β2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of ≥260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308μg/L vs 146μg/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to β2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; ≥1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry profiling is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.