Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy; Division of Hematology, Papa Giovanni XXIII Hospital, Bergamo, Italy.
Am J Hematol. 2014 Jun;89(6):588-90. doi: 10.1002/ajh.23694. Epub 2014 Mar 3.
In patients not meeting the required hematocrit (HCT) or hemoglobin (Hb) thresholds according to BCSH and the WHO diagnostic criteria, the diagnosis of masked polycythemia vera (mPV) has been proposed. A comparison of HCT or Hb values with the expression of JAK2V617F, JAK2 exon 12, and CALR mutations in strictly WHO-defined 257 overt PV and 140 mPV (59 mPV according to BCSH) and 397 patients with essential thrombocythemia (ET) was performed. Hb and HCT thresholds of mPV patients were significantly higher than JAK2V617F ET (P < 0.0001). The best cut-off for Hb to discriminate JAK2-mutated ET from PV was 16.5 g/dL for males and 16.0 g/dL for females. For HCT, this was 49% in males and 48% in females. The proportion of patients correctly classified as ET or PV when regarding Hb or HCT levels was 95% in males and 93% in females and 94% in both males and females, respectively.
在不符合 BCSH 和世界卫生组织诊断标准所要求的血细胞比容（HCT）或血红蛋白（Hb）阈值的患者中，已经提出了隐匿性真性红细胞增多症（mPV）的诊断。将 HCT 或 Hb 值与严格按照世界卫生组织定义的 257 例显性真性红细胞增多症（PV）和 140 例隐匿性真性红细胞增多症（BCSH 定义的 59 例 mPV）和 397 例原发性血小板增多症（ET）中 JAK2V617F、JAK2 外显子 12 和 CALR 突变的表达进行了比较。mPV 患者的 Hb 和 HCT 阈值明显高于 JAK2V617F ET（P<0.0001）。用于区分 JAK2 突变 ET 与 PV 的 Hb 最佳截断值为男性 16.5 g/dL，女性 16.0 g/dL。对于 HCT，男性为 49%，女性为 48%。当分别以 Hb 或 HCT 水平作为参考时，男性正确分类为 ET 或 PV 的患者比例为 95%，女性为 93%，男女均为 94%。
