Increased CD11b neutrophil expression in Budd-Chiari syndrome or portal vein thrombosis secondary to polycythaemia vera

Abstract Myeloproliferative neoplasms (MPNs) are the most common cause of Budd-Chiari syndrome (BCS) and nonmalignant, noncirrhotic portal vein thrombosis (PVT). In this meta-analysis, we determined the prevalence of MPNs and their subtypes as well as JAK2V617F and its diagnostic role in these uncommon disorders. MEDLINE and EMBASE databases were searched. Prevalence of MPNs, JAK2V617F, and MPN subtypes were calculated using a random-effects model. A total of 1062 BCS and 855 PVT patients were included. In BCS, mean prevalence of MPNs and JAK2V617F was 40.9% (95% CI, 32.9%-49.5%) and 41.1% (95% CI, 32.3%-50.6%), respectively. In PVT, mean prevalence of MPNs and JAK2V617F was 31.5% (95% CI, 25.1%-38.8%) and 27.7% (95% CI, 20.8%-35.8%), respectively. JAK2V617F and MPNs were more frequent in BCS compared with PVT (P = .03 and P = .09, respectively). Polycythemia vera was more prevalent in BCS than in PVT (P = .001). JAK2V617F screening in splanchnic vein thrombosis (SVT) patients without typical hematologic MPN features identified MPN in 17.1% and 15.4% of screened BCS and PVT patients, respectively. These results demonstrate a high prevalence of MPNs and JAK2V617F in SVT patients and show differences in underlying etiology between these disorders. Furthermore, these results validate routine inclusion of JAK2V617F in the diagnostic workup of SVT patients.

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Prevalence of paroxysmal nocturnal hemoglobinuria in Chinese patients with Budd-Chiari syndrome or portal vein thrombosis

Journal of Gastroenterology and Hepatology ◽

10.1111/j.1440-1746.2012.07282.x ◽

2012 ◽

Vol 28 (1) ◽

pp. 148-152 ◽

Cited By ~ 25

Author(s):

Xingshun Qi ◽

Chuangye He ◽

Guohong Han ◽

Zhanxin Yin ◽

Feifei Wu ◽

...

Keyword(s):

Portal Vein ◽

Portal Vein Thrombosis ◽

Paroxysmal Nocturnal Hemoglobinuria ◽

Chinese Patients ◽

Budd Chiari Syndrome ◽

Vein Thrombosis ◽

Chiari Syndrome

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Prevalence of portal vein thrombosis in Egyptian patients with Budd-Chiari syndrome

Indian Journal of Gastroenterology ◽

10.1007/s12664-014-0483-x ◽

2014 ◽

Vol 33 (5) ◽

pp. 489-491 ◽

Cited By ~ 1

Author(s):

Mohammed Amin Sakr ◽

Nadia Abdelkader ◽

Hany Dabbous ◽

Ahmed Eldorry

Keyword(s):

Portal Vein ◽

Portal Vein Thrombosis ◽

Budd Chiari Syndrome ◽

Vein Thrombosis ◽

Egyptian Patients ◽

Chiari Syndrome

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Pregnancy outcomes in women with Budd-Chiari syndrome or portal vein thrombosis - A multicentre retrospective cohort study

10.22541/au.162126800.05949743/v1 ◽

2021 ◽

Author(s):

Hanke Wiegers ◽

Eva N. Hamulyák ◽

Stefanie E. Damhuis ◽

Jack R. Duuren ◽

Sarwa Darwish Murad ◽

...

Keyword(s):

Cohort Study ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Live Birth ◽

Retrospective Cohort Study ◽

Retrospective Cohort ◽

Budd Chiari Syndrome ◽

Live Births ◽

Vein Thrombosis ◽

Chiari Syndrome

Objective: to evaluate current practice and outcomes of pregnancy in women previously diagnosed with Budd-Chiari syndrome and/or portal vein thrombosis, with and without concomitant portal hypertension. Design and setting: multicentre retrospective cohort study between 2008-2021 Population: Women who conceived in the predefined period after the diagnosis of Budd-Chiari syndrome and/or portal vein thrombosis Methods and main outcome measures: We collected data on diagnosis and clinical features. The primary outcomes were maternal mortality and live birth rate. Secondary outcomes included maternal, neonatal and obstetric complications. Results: Forty-five women (12 Budd-Chiari syndrome, 33 portal vein thrombosis; 76 pregnancies) were included. Underlying prothrombotic disorders were present in 23 of 45 women (51%). Thirty-eight women (84%) received low-molecular-weight heparin during pregnancy. Of 45 first pregnancies, 11 (24%) ended in pregnancy loss and 34 (76%) resulted in live birth of which 27 at term age (79% of live births and 60% of pregnancies). No maternal deaths were observed, one woman developed pulmonary embolism during pregnancy and two women (4%) had variceal bleeding requiring intervention. Conclusions: The high number of term live births (79%) and lower than expected risk of pregnancy-related maternal and neonatal morbidity in our cohort suggest that Budd-Chiari syndrome and/or portal vein thrombosis should not be considered as an absolute contra-indication for pregnancy. Individualized, nuanced counselling and a multidisciplinary pregnancy surveillance approach are essential in this patient population.

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