Division of Hematology and Medical Oncology, Department of Medicine.
Blood. 2013 Sep 12;122(11):1881-6. doi: 10.1182/blood-2013-06-508416. Epub 2013 Jul 30.
We prospectively evaluated the accuracy of the 2007 World Health Organization (WHO) criteria for diagnosing polycythemia vera (PV), especially in "early-stage" patients. A total of 28 of 30 patients were diagnosed as PV owing to an elevated Cr-51 red cell mass (RCM), JAK2 positivity, and at least 1 minor criterion. A total of 18 PV patients did not meet the WHO criterion for an increased hemoglobin value and 8 did not meet the WHO criterion for an increased hematocrit value. Bone marrow morphology was very valuable for diagnosis. Low serum erythropoietin (EPO) values were specific for PV, but normal EPO values were found at presentation (20%). We recommend revision of the WHO criteria, especially to distinguish early-stage PV from essential thrombocythemia. Major criteria remain JAK2 positivity and increased red cell volume, but Cr-51 RCM is mandatory for patients who do not meet the defined elevated hemoglobin or hematocrit value (>18.5 g/dL and 60% in men and >16.5 g/dL and 56% in women, respectively). Minor criteria remain bone marrow histology or a low serum EPO value. For patients with a normal EPO value, marrow examination is mandatory for diagnostic confirmation. Because the therapies for myeloproliferative disorders differ, our data have major clinical implications.
我们前瞻性地评估了 2007 年世界卫生组织（WHO）诊断真性红细胞增多症（PV）的标准的准确性，特别是在“早期”患者中。由于 Cr-51 红细胞容量（RCM）升高、JAK2 阳性和至少 1 个次要标准，共有 30 名患者中的 28 名被诊断为 PV。共有 18 名 PV 患者不符合 WHO 血红蛋白值升高的标准，8 名患者不符合 WHO 血细胞比容值升高的标准。骨髓形态学对诊断非常有价值。低血清促红细胞生成素（EPO）值对 PV 具有特异性，但在发病时发现正常的 EPO 值（20%）。我们建议修订 WHO 标准，特别是要区分早期 PV 和原发性血小板增多症。主要标准仍然是 JAK2 阳性和红细胞体积增加，但对于不符合定义的血红蛋白或血细胞比容值升高的患者（男性分别为>18.5 g/dL 和 60%，女性分别为>16.5 g/dL 和 56%），Cr-51 RCM 是强制性的。次要标准仍然是骨髓组织学或低血清 EPO 值。对于 EPO 值正常的患者，骨髓检查是诊断确认的强制性要求。由于骨髓增生性疾病的治疗方法不同，我们的数据具有重要的临床意义。
