Ph-Negative Chronic Myeloproliferative Neoplasm (Primary Myelofibrosis) – as One of the Reasons of the Budd-Chiari Syndrome

Dear Editor, Valla and his team pioneered the thorough search for latent myeloproliferative neoplasms (MPN) in patients with splanchnic thrombosis. They noticed that “primary myeloproliferative disorder, often without peripheral blood changes, is a major cause of hepatic vein thrombosis”. Valla provided precious guidelines for anticoagulant therapy suggesting to start anticoagulation as early as possible, since it is much more effective in preventing extension to or from the portal vein than in inducing complete recanalization of extrahepatic portal vein obstruction. The problem of latent or evident MPN in young people with splanchnic venous thrombosis also was investigated by Teofili L, De Stefano V, Leone G. Special attention was paid to the proble Splanchnic Vein Thrombosis and clinical course of MPN []. The main conclusion of their research was that the leading cause of splanchnic vein thrombosis are MPN, which are diagnosed , even if a latent form, in 50% of patients with Budd-Chiari syndrome (BCS), furthermore deficiency of natural anticoagulants can favor the insurgence of BCS. Our case further shows that in absence of classical criteria and molecular diagnostic tools the diagnosis of MPN can be overlooked and the splanchnic www.mjhid.org ISSN 2035-3006