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Tryptase and histamine metabolites as diagnostic indicators of indolent systemic mastocytosis without skin lesions

机译:类胰蛋白酶和组胺代谢产物可作为无皮肤病变的轻度全身性肥大细胞增多症的诊​​断指标

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Background: Risk indicators of indolent systemic mastocytosis (ISM) in adults with clinical suspicion of ISM without accompanying skin lesions [urticaria pigmentosa (UP)] are lacking. This study aimed at creating a decision tree using clinical characteristics, serum tryptase, and the urinary histamine metabolites methylimidazole acetic acid (MIMA) and methylhistamine (MH) to select patients for bone marrow investigations to diagnose ISM. Methods: Retrospective data analysis of all adults, in whom bone marrow investigations were performed to diagnose ISM, was carried out. Results: In total, 142 patients were included. SM was absent in all 44 patients with tryptase <10 μg/l, in 45 of 98 (46%) patients with tryptase ≥10 μg/l and in 18 of 52 patients (35%) with tryptase >20 μg/l. Above 43 μg/l, all patients had ISM (n = 11). Male gender, insect venom anaphylaxis as presenting symptom, tryptase, MIMA, and MH were independent ISM predictors. If tryptase was ≥10 μg/l, the diagnostic accuracy of MIMA and MH was high (areas under the ROC curve 0.92). Conclusions: In suspected patients without UP, the ISM risk is very low (if present at all) if tryptase is <10 lg/l. If tryptase is ≥10 μg/l, this risk depends on MIMA and MH, being low if these are normal, but high if these are elevated. Male gender and insect venom anaphylaxis are additional risk indicators. We recommend refraining from bone marrow examinations in suspected patients without UP if tryptase is <10 μg/l. Our results question the reliability of the minor diagnostic World Health Organization criterion of tryptase >20 μg/l.
机译:背景:缺乏临床上怀疑患有ISM而没有伴随皮肤损害[色素性荨麻疹(UP)]的成年人,其惰性系统性肥大细胞增多症(ISM)的风险指标缺乏。这项研究旨在利用临床特征,血清类胰蛋白酶和尿液中的组胺代谢产物甲基咪唑乙酸(MIMA)和甲基组胺(MH)来创建决策树,以选择要进行骨髓检查以诊断ISM的患者。方法:对所有成人进行回顾性数据分析,其中进行了骨髓检查以诊断ISM。结果:总共包括142例患者。胰蛋白酶<10μg/ l的所有44例患者中,SM≥10μg/ l的98例中的45(46%)患者和胰蛋白酶> 20μg/ l的52例中的18例(35%)不存在SM。高于43μg/ l,所有患者均患有ISM(n = 11)。男性,表现为症状的昆虫毒物过敏,类胰蛋白酶,MIMA和MH是ISM的独立预测因子。如果类胰蛋白酶≥10μg/ l,则MIMA和MH的诊断准确性较高(ROC曲线下的面积为0.92)。结论:如果胰蛋白酶<10 lg / l,则怀疑没有UP的患者的ISM风险非常低(如果有的话)。如果类胰蛋白酶≥10μg/ l,则此风险取决于MIMA和MH;如果正常,则风险较低;如果升高,则风险较高。男性性别和昆虫毒液过敏是其他风险指标。如果胰蛋白酶<10μg/ l,我们建议不要对没有UP的可疑患者进行骨髓检查。我们的结果质疑胰蛋白酶> 20μg/ l的次要诊断性世界卫生组织标准的可靠性。

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