Prevalence of CALR mutations in splanchnic vein thrombosis: A systematic review and meta-analysis. Issue 167 (July 2018)

• Record Type: Journal Article
• Authors: Li, Miaomiao; De Stefano, Valerio; Song, Tingxue; Zhou, Xinmiao; Guo, Zeqi; Zhu, Jia; Qi, Xingshun
• Is Part Of: Thrombosis research. Issue 167(2018)
• Page Start: 96
• Page End: 103
• Publication Date: 2018-07
• Subjects: CALR -- Portal vein -- Myeloproliferative neoplasm -- JAK2 -- Hepatic vein; Thrombosis -- Periodicals; 616.135
• DOI: 10.1016/j.thromres.2018.05.007
• Languages: English
• ISSNs: 0049-3848

Abstract

Abstract: Background: 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. Aim: 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. Methods: 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. Results: 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. Conclusion: 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. Highlights: There is a very low prevalence of Calreticulin mutations in general patients with splanchnic vein thrombosis (0%-4.88%) There is a remarkable variation in the prevalence of Calreticulin mutations according to the status of myeloproliferative neoplasms and JAK2V617F mutation The pooled proportion of Calreticulin mutations in patients with splanchnic vein thrombosis and myeloproliferative neoplasms but without JAK2V617F mutation was 15.16% Calreticulin mutations are rarely observed in patients with splanchnic vein thrombosis and screening should be selective.