A latent form of essential thrombocythemia presenting asportal cavernoma

Abstract

Essential thrombocythemia is frequently associated with abdominal thrombotic complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype. In the presented case report, the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism. The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.

Portal Hypertension - Causes and Complications

“…In non-cirrhotic and non-tumoral PVT cases in the West, MPD (i.e. polycythaemia vera, essential thrombocythaemia and myelofibrosis) with a combination of several prothrombotic factors constitute the most common identifiable cause with an estimated prevalence of 30%-60% (Cai et al, 2009;Kiladjian et al, 2006;Kutti & Ridell, 2001). In another study, a myeloproliferative disorder (MPD) was found in 37% of patients with non-cirrhotic non-malignant PVT (Kiladjian et al, 2006).…”

“…The annual incidence of myeloproliferative syndrome is 2,1-3,5 per 100000 peoples (Kutti & Ridell, 2001). From the four entities comprising the myeloproliferative syndrome according to the FAB classification (essential thrombocythemia, polycythaemia vera, chronic myelogenous leukemia and idiopathic myelofibrosis), essential thrombocythemia is considered the most frequent disease with an annual incidence of 0,7-2,5 per 100000 peoples (Cai et al, 2009;Girodon et al, 2009;Rollison et al, 2008). The transformation to acute myelogenous leukemia is recorded in 0,6-5% of patients and the overall 10-year survival rate is 64-80% (Fenaux et al, 1990).…”

“…Once the liver blood supply decreases significantly, the compensatory mechanism is activated and collaterals begin to form within a few days after the obstruction and organize into a cavernous transformation in 3-5 weeks. (Cai, 2009) Based on a case previously communicated by us -(A case of portal cavernoma -associated thrombocythemia -A. Rosu, C. Searpe, V. Sbarcea, Z. Stoica, M. Popescu. J Gastrointestin Liver Dis 2007 16(1): 97-100), a review of the published English literature was performed using PubMed ® (http://www.ncbi.nlm.nih.gov/PubMed) and Medline database.…”

Portal Vein Thrombosis with Cavernous Transformation in Myeloproliferative Disorders: Review Update

“…In non-cirrhotic and non-tumoral PVT cases in the West, MPD (i.e. polycythaemia vera, essential thrombocythaemia and myelofibrosis) with a combination of several prothrombotic factors constitute the most common identifiable cause with an estimated prevalence of 30%-60% (Cai et al, 2009;Kiladjian et al, 2006;Kutti & Ridell, 2001). In another study, a myeloproliferative disorder (MPD) was found in 37% of patients with non-cirrhotic non-malignant PVT (Kiladjian et al, 2006).…”

“…From the four entities comprising the myeloproliferative syndrome according to the FAB classification (essential thrombocythemia, polycythaemia vera, chronic myelogenous leukemia and idiopathic myelofibrosis), essential thrombocythemia is considered the most frequent disease with an annual incidence of 0,7-2,5 per 100000 peoples (Cai et al, 2009;Girodon et al, 2009;Rollison et al, 2008). The transformation to acute myelogenous leukemia is recorded in 0,6-5% of patients and the overall 10-year survival rate is 64-80% (Fenaux et al, 1990).…”