Polycythemia Vera Complicated by Portal Vein Thrombosis and Budd-Chiari Syndrome:

2021 ◽  
Vol 10 (02) ◽  
pp. 163-165
Author(s):  
Nabeela Iqbal ◽  
Syed Khalid Shah ◽  
Shamima Haneef

Polycythemia vera is a medical condition characterized by raised hematocrit. Owing to increased viscosity, the blood flow in the vessels become sluggish leading to the clinical features of polycythemia such as headache, blurring of vision, red skin, dizziness, raised blood pressure, itching and more serious medical events like vaso occlusion, thrombosis and strokes. In this case report, polycythemia vera presenting unusually with heamatemesis, melena and abdominal distension. Physical examination of this case revealed massive ascites with dilated veins around the umbilicus. The diagnosis of polycythemia vera complicated by Budd Chiari Syndrome and Portal Vein Thrombosis was made. Patients with polycythemia vera are at risk of vaso occlusive sequelea like portal vein thrombosis and Budd chiari syndrome

2013 ◽  
pp. 196-205
Author(s):  
Aurelio Seidita ◽  
Delia Sprini ◽  
Accursia Bongiovì ◽  
Tiziana Catalano ◽  
Filippo Barbiera ◽  
...  

Budd-Chiari syndrome may be defined as a heterogeneous group of vascular disorders characterized by obstruction of hepatic venous return to the level of hepatic venules, supra-hepatic veins, inferior vena cava or right atrium. The main cause of this syndrome is represented by myeloproliferative diseases and, in particular, by polycythemia vera. The latter may cause multiple splanchnic thrombosis, including portal vein thrombosis, particularly important for its clinical outcomes (ascites, collateral vessels genesis, etc.). We report 2 cases of a Budd-Chiari syndrome induced by polycythemia vera characterized by an abnormal clinical onset, both as regards subjects’ age (29 and 39 years old, respectively) and set of symptoms, signs and laboratory data. After a complete clinical, instrumental and genetic diagnosis, the patients were treated with combined therapy, using acetylsalicylic acid and hydroxyurea. The therapy proved successful and patients are still in follow up in our institution. Polycythemia vera should be suspected in patients affected with portal vein thrombosis and Budd-Chiari syndrome even if its clinical onset might be unusual. Every effort should be made to make a correct and early diagnosis in order to start appropriate therapy as soon as possible and to prevent patients from useless diagnostic and therapeutic treatments.


2004 ◽  
Vol 121 (6) ◽  
pp. 844-847 ◽  
Author(s):  
Maitreyee Bhattacharyya ◽  
Govind Makharia ◽  
M. Kannan ◽  
R.P.H. Ahmed ◽  
P.K. Gupta ◽  
...  

2009 ◽  
Vol 13 (1) ◽  
pp. 127-144 ◽  
Author(s):  
Paulo Lisboa Bittencourt ◽  
Cláudia Alves Couto ◽  
Daniel Dias Ribeiro

2004 ◽  
Vol 124 (3) ◽  
pp. 329-335 ◽  
Author(s):  
Alberto Alvarez-Larrán ◽  
Juan Carlos García-Pagán ◽  
Juan G. Abraldes ◽  
Eduardo Arellano ◽  
Juan Carlos Reverter ◽  
...  

2004 ◽  
Vol 78 (1) ◽  
pp. 333-334 ◽  
Author(s):  
George K Anagnostopoulos ◽  
George Margantinis ◽  
Panagiotis Kostopoulos ◽  
Glyceria Papadopoulou ◽  
Athanassios Roulias ◽  
...  

2016 ◽  
Vol 111 ◽  
pp. S815-S816
Author(s):  
Yezaz A. Ghouri ◽  
Akhil V. Shenoy ◽  
Heather L. Stevenson ◽  
Shehzad Merwat

Blood ◽  
2012 ◽  
Vol 120 (25) ◽  
pp. 4921-4928 ◽  
Author(s):  
Jasper H. Smalberg ◽  
Lidia R. Arends ◽  
Dominique C. Valla ◽  
Jean-Jacques Kiladjian ◽  
Harry L. A. Janssen ◽  
...  

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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