Khurana Harshit, Lakshman Praveen, Kumar Kishore, Jain Arihant
1Medical Division, Command Hospital Air Force (CHAF), Bangalore, Karnataka India.
2Department of Internal Medicine (Clinical Hematology Division), Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Indian J Hematol Blood Transfus. 2020 Jan;36(1):187-191. doi: 10.1007/s12288-019-01155-0. Epub 2019 Jul 8.
Referrals for evaluation of polycythemia cases have increased since the hemoglobin (Hb) thresholds for diagnosis of Polycythemia Vera (PV) have been lowered by WHO. The current study enrolled patients of age > 18 years from the Indian Armed Forces or their family members with polycythemia from November 2016 to October 2018. After exclusion of secondary causes, 49 patients were diagnosed as Primary Erythrocytosis (PE). The patients were classified into two groups: PV and Idiopathic Erythrocytosis (IE) and a systematic comparison of clinical and laboratory features of the two groups was done. The prevalence of PV in PE was 20.4% (10 of 49) while the rest 39 (79.6%) had IE. Seven PV patients had mutation, one had mutation, and two were JAK2 negative PV. Nine of 10 (90%) PV patients had Hb > 18.5 g/dl, while only 21 of 39 (53.8%) IE patients had Hb > 18.5 g/dl ( = 0.06). None of the JAK2 mutated patients had Hb < 18.5 g/dl. We conclude that PV is more prevalent in patients of PE with Hb > 18.5 g/dl. Most patients with Hb between 16.5-18.5 g/dl would still be classified as IE. We advocate the need for further studies evaluating the utility of investigating all patients of PE with the revised WHO Hb threshold as well as studies on genetic profile of IE patients from India.
自从世界卫生组织（WHO）降低真性红细胞增多症（PV）的血红蛋白（Hb）诊断阈值以来，转诊来评估红细胞增多症病例的数量有所增加。本研究纳入了2016年11月至2018年10月期间来自印度武装部队或其家庭成员中年龄大于18岁的红细胞增多症患者。排除继发原因后，49例患者被诊断为原发性红细胞增多症（PE）。这些患者被分为两组：PV组和特发性红细胞增多症（IE）组，并对两组的临床和实验室特征进行了系统比较。PE患者中PV的患病率为20.4%（49例中的10例），其余(79.6%)为IE。7例PV患者有JAK2突变，1例有CALR突变，2例为JAK2阴性PV。10例PV患者中有9例（90%）Hb>18.5 g/dl，而39例IE患者中只有21例（53.8%）Hb>18.5 g/dl（P=0.06）。JAK2突变的患者中没有Hb<18.5 g/dl的。我们得出结论，Hb>18.5 g/dl的PE患者中PV更为常见。大多数Hb在16.5 - 18.5 g/dl之间的患者仍将被归类为IE。我们主张需要进一步研究评估按照WHO修订的Hb阈值对所有PE患者进行检查的实用性，以及对来自印度的IE患者的基因谱进行研究。
