Kourie Hampig Raphael, Ameye Lieveke, Paesmans Marianne, Bron Dominique
Hematology Department, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium.
Data Centre, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium.
Clin Lymphoma Myeloma Leuk. 2016 May;16(5):264-8. doi: 10.1016/j.clml.2016.01.009. Epub 2016 Feb 6.
Next to janus kinase 2 (JAK2) and myeloproliferative leukemia protein, calreticulin (CALR) is a recently discovered mutation present in > 20% of patients diagnosed with primary myelofibrosis (PMF).
Six studies published from December 2013 to December 2014 met the inclusion criteria for the present meta-analysis: 2 of an Asian and 4 of a non-Asian population. We assessed the biologic characteristics at diagnosis and investigated overall survival. The analyses were stratified by ethnic origin (Asian vs. non-Asian).
A total of 816 patients with the JAK2 mutation and 307 patients with the CALR mutation were included. The patients with the JAK2 mutation were older than those with the CALR mutation, and no statistically significant difference was noted in the gender distribution of the patients with PMF with the JAK2 versus CALR mutation. Patients with JAK2-mutated PMF had a higher white blood cell count, but no statistically significant evidence was found for a difference in the platelet count or hemoglobin level. No difference was found in thrombosis risk or acute leukemic transformation in those 2 populations. Major differences were found between the Asian and non-Asian populations. The difference in characteristics between JAK2 and CALR was larger in the Asian population than in the non-Asian population (P = .007). Also, in the non-Asian population, those with JAK2 mutation had lower platelet counts than the Asians (P = .06). In the non-Asian population, the patients diagnosed with JAK2-positive PMF had worse overall survival than the patients with CALR-positive PMF, with a combined hazard ratio of 2.43 (95% confidence interval, 1.83-3.22).
The results of the present meta-analysis have confirmed the role of the CALR mutation in the diagnosis of, and as a prognostic tool for, PMF. Our results suggest that patients with the CALR mutation will have better overall survival than patients with the JAK2 mutation in a non-Asian population.
除了 Janus 激酶 2（JAK2）和骨髓增殖性白血病蛋白外，钙网蛋白（CALR）是最近在超过 20%被诊断为原发性骨髓纤维化（PMF）的患者中发现的一种突变。
2013 年 12 月至 2014 年 12 月发表的六项研究符合本荟萃分析的纳入标准：2 项来自亚洲人群，4 项来自非亚洲人群。我们评估了诊断时的生物学特征并调查了总生存期。分析按种族来源（亚洲人与非亚洲人）进行分层。
共纳入 816 例 JAK2 突变患者和 307 例 CALR 突变患者。JAK2 突变患者比 CALR 突变患者年龄更大，JAK2 突变与 CALR 突变的 PMF 患者在性别分布上无统计学显著差异。JAK2 突变的 PMF 患者白细胞计数较高，但在血小板计数或血红蛋白水平方面未发现有统计学显著差异的证据。这两组人群在血栓形成风险或急性白血病转化方面未发现差异。亚洲人群和非亚洲人群之间存在主要差异。JAK2 和 CALR 在亚洲人群中的特征差异大于非亚洲人群（P = 0.007）。此外，在非亚洲人群中，JAK2 突变患者的血小板计数低于亚洲人（P = 0.06）。在非亚洲人群中，诊断为 JAK2 阳性 PMF 的患者总生存期比 CALR 阳性 PMF 的患者更差，合并风险比为 2.43（95%置信区间，1.83 - 3.22）。
本荟萃分析的结果证实了 CALR 突变在 PMF 诊断及作为预后工具方面的作用。我们的结果表明，在非亚洲人群中，CALR 突变患者的总生存期将优于 JAK2 突变患者。
