Case-control cohort study of patients' perceptions of disability in mastocytosis.
Case-control cohort study of patients' perceptions of disability in mastocytosis.
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DOI：
10.1371/journal.pone.0002266
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发表时间：
2008-05-28
期刊：
影响因子：
3.7
通讯作者：
Moussy, Alain
中科院分区：
文献类型：
--
作者：
Hermine, Olivier;Lortholary, Olivier;Leventhal, Phillip S.;Catteau, Adeline;Soppelsa, Frederique;Baude, Cedric;Cohen-Akenine, Annick;Palmerini, Fabienne;Hanssens, Katia;Yang, Ying;Sobol, Hagay;Fraytag, Sylvie;Ghez, David;Suarez, Felipe;Barete, Stephane;Casassus, Philippe;Sans, Beatrice;Arock, Michel;Kinet, Jean Pierre;Dubreuil, Patrice;Moussy, Alain
肥大细胞增多症的惰性形式占所有病例的90%以上，但症状的类型和严重程度及其对生活质量的影响尚未得到充分研究。因此，我们进行了一项病例对照队列研究，以检查自我报告的残疾和症状对肥大细胞增多症患者生活质量的影响。2004年，法国363名肥大细胞增多症患者和90名对照者被要求对他们的总体残疾（OPA评分）和38种症状的严重程度进行评分。后者用于计算综合评分（AFIRMM评分）。在363名受访者中，有262名参与了正在进行的病理生理学研究，因此可以获得以下数据：世界卫生组织分类、身心残疾的标准衡量标准、D816 V KIT突变的存在和血清类胰蛋白酶水平。平均OPA和AFIRMM评分和残疾的标准措施表明，大多数肥大细胞增多症患者患有残疾，由于这种疾病。令人惊讶的是，根据疾病分类或D816 V KIT突变的存在或不存在或血清类胰蛋白酶水平升高（≥20 ng/mL），患者的可测量和感知残疾没有差异。此外，38个AFIRMM症状中的32个在患者中比对照组更常见，但根据疾病分类、D816 V突变的存在或血清类胰蛋白酶水平没有实质性差异。基于这些结果和治疗的目的，我们建议首先将肥大细胞增多症分为侵袭性或惰性，然后根据患者感知症状的严重程度及其对生活质量的影响对惰性肥大细胞增多症进行分类。此外，肥大细胞增多症患者出现的症状和残疾似乎比以前认为的更多，因此肥大细胞增多症可能诊断不足，并且惰性形式的肥大细胞增多症的症状可能更多地是由于介质的全身释放而不是肥大细胞负荷。
Indolent forms of mastocytosis account for more than 90% of all cases, but the types and type and severity of symptoms and their impact on the quality of life have not been well studied. We therefore performed a case-control cohort study to examine self-reported disability and impact of symptoms on the quality of life in patients with mastocytosis. In 2004, 363 mastocytosis patients and 90 controls in France were asked to rate to their overall disability (OPA score) and the severity of 38 individual symptoms. The latter was used to calculate a composite score (AFIRMM score). Of the 363 respondents, 262 were part of an ongoing pathophysiological study so that the following data were available: World Health Organization classification, standard measures of physical and psychological disability, existence of the D816V KIT mutation, and serum tryptase level. The mean OPA and AFIRMM scores and the standard measures of disability indicated that most mastocytosis patients suffer from disabilities due to the disease. Surprisingly, the patient's measurable and perceived disabilities did not differ according to disease classification or presence or absence of the D816V KIT mutation or an elevated (≥20 ng/mL) serum tryptase level. Also, 32 of the 38 AFIRMM symptoms were more common in patients than controls, but there were not substantial differences according to disease classification, presence of the D816V mutation, or the serum tryptase level. On the basis of these results and for the purposes of treatment, we propose that mastocytosis be first classified as aggressive or indolent and that indolent mastocytosis then be categorized according to the severity of patients' perceived symptoms and their impact on the quality of life. In addition, it appears that mastocytosis patients suffer from more symptoms and greater disability than previously thought, that mastocytosis may therefore be under-diagnosed, and that the symptoms of the indolent forms of mastocytosis might be due more to systemic release of mediators than mast cell burden.
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影响因子：
20.3
作者：
Sperr, WR;Jordan, JH;Valent, P
通讯作者：
Valent, P
影响因子：
2.8
作者：
Féger, F;Dumas, AR;Arock, M
通讯作者：
Arock, M
影响因子：
13.8
作者：
Proelss, Julia;Wenzel, Joerg;Bauer, Ralf
通讯作者：
Bauer, Ralf
DOI：
10.1093/jnci/85.5.365
发表时间：
1993-03-03
期刊：
JOURNAL OF THE NATIONAL CANCER INSTITUTE
影响因子：
--
作者：
AARONSON, NK;AHMEDZAI, S;TAKEDA, F
通讯作者：
TAKEDA, F
影响因子：
3.3
作者：
ROGERS, MP;BLOOMINGDALE, K;AUSTEN, KF
通讯作者：
AUSTEN, KF