内脏静脉血栓形成(SVT)中钙网蛋白(CALR)突变的患病率在研究中有所不同。常规筛查CALR突变在SVT患者中的作用仍存在争议。
根据不同类型合成CALR突变的患病率(即,Budd-Chiari综合征[BCS]和门静脉血栓形成[PVT])和特征(即,伴和不伴骨髓增殖性肿瘤[MPNs]和JAK2V617F突变)的SVT患者。
通过PubMed和Embase数据库检索符合条件的研究。根据STROBE检查表评估研究质量。通过使用随机效应模型汇集CALR突变的比例。计算异质性和发表偏倚。
共收录了11篇论文。研究质量中等到高。CALR突变的合并比例为1.21%,1.41%,和1.59%的SVT,BCS,和PVT患者,分别为1.52%、1.03%,在这些没有JAK2V617F突变的患者中,1.82%,分别为3.71%,2.79%,在这些患有MPN的患者中,有7.87%,分别为15.16%,17.22%,在这些患有MPN但没有JAK2V617F突变的患者中,为31.44%,分别。只有荟萃分析检查MPN但无JAK2V617F突变的BCS患者中CLAR突变的患病率显示出统计学上显著的异质性。仅在对无JAK2V617F突变的SVT患者CALR突变患病率进行的荟萃分析中发现有统计学意义的发表偏倚。
CALR突变的筛查可能在排除JAK2V617F突变的MPN概率较高的SVT患者中发挥作用。
The prevalence of calreticulin (
CALR) mutations in splanchnic vein thrombosis (SVT) varies among studies. The role of routine screening for
CALR mutations in SVT patients remains a debate.
To synthesize the prevalence of
CALR mutations according to the different types (i.e., Budd-Chiari syndrome [BCS] and portal vein thrombosis [PVT]) and characteristics (i.e., with and without myeloproliferative neoplasms [MPNs] and JAK2V617F mutation) of SVT patients.
Eligible studies were searched by the PubMed and Embase databases. The study quality was assessed according to the STROBE checklist. The proportion of
CALR mutations was pooled by using a random-effects model. The heterogeneity and publication bias were calculated.
Eleven papers were included. The study quality was moderate to high. The pooled proportion of CALR mutations was 1.21%, 1.41%, and 1.59% in SVT, BCS, and PVT patients, respectively; 1.52%, 1.03%, and 1.82% in these patients without JAK2V617F mutation, respectively; 3.71%, 2.79%, and 7.87% in these patients with MPN, respectively; and 15.16%, 17.22%, and 31.44% in these patients with MPN but without JAK2V617F mutation, respectively. Only the meta-analysis examining the prevalence of CLAR mutations in BCS patients with MPN but without the JAK2V617F mutation showed statistically significant heterogeneity. Statistically significant publication bias was seen only in the meta-analysis examining the prevalence of CALR mutations in SVT patients without the JAK2V617F mutation.
Screening for
CALR mutations may have a role in SVT patients with a high probability of MPN in whom the JAK2V617F mutation has been excluded.