骨髓增生异常综合征诊断准确性分级系统的建议

Akira Matsuda , Itsuro Jinnai , Yasushi Miyazaki , Masao Tomonaga
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引用次数: 9

摘要

尽管最近在细胞遗传学和分子研究方面取得了进展,但用于骨髓增生异常综合征(MDS)诊断的通用生物标志物仍然缺乏。仅凭形态学诊断MDS并不容易,特别是在患有<骨髓中有5%的细胞(BM),核型正常。因此,可能会出现误诊和观察者之间的不一致。为了解决这些问题,我们提出了一个MDS诊断准确率分级系统。MDS的诊断准确性分为“明确”、“可能”和“可能”,此外还有“意义不确定的特发性细胞减少症(ICUS)”。诊断准确性的分级标准是(1)BM中母细胞的频率,(2)发育不良的等级(高、中、低),以及(3)细胞遗传学的分裂(异常、正常或未知)。为了对发育不良进行定量形态学评估,我们将形态发育不良的改变分为高度特异性的A类(伪pelger - huet异常、中性粒细胞脱颗粒、微巨核细胞、和环状sideroblasts)和不太具体的B类(除A类外的发育不良)。我们认为,通过使用我们的分级系统,并在适当的时间间隔对被分配到“可能”或“ICUS”类别的患者重复BM检查,可以减少诊断问题,这将使MDS类别的模糊界限更加清晰。
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Proposals for a Grading System for Diagnostic Accuracy of Myelodysplastic Syndromes

Despite recent advances in cytogenetics and molecular research, universal biomarkers for the diagnosis of the myelodysplastic syndromes (MDS) are still lacking. It is not easy to diagnose MDS by morphology alone, particularly in patients with < 5% blasts in the bone marrow (BM) and normal karyotype. Therefore, the possibility of misdiagnosis and discordance among observers can occur. In order to resolve these problems, we propose a grading system for diagnostic accuracy of MDS. The diagnostic accuracy of MDS is graded into “definite,” “probable,” or “possible” in addition to “idiopathic cytopenia(s) of uncertain significance (ICUS).” The criteria of grading for diagnostic accuracy are a combination of (1) the frequency of blasts in BM, (2) grade of dysplasia (high, intermediate, or low), and (3) division of cytogenetics (abnormal, normal, or unknown). For quantitative morphologic evaluation of dysplasias, we classified morphologic dysplastic changes into highly specific category A (pseudo–Pelger-Huet anomaly, degranulation of neutrophils, micromegakaryocytes, and ringed sideroblasts) and less specific category B (dysplasias other than those in category A). We believe that diagnostic problems would be reduced by using our grading system and repeating BM examination at suitable intervals for patients who are allocated into the “possible” or “ICUS” categories, and this will make the vague margin of MDS category clearer.

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