%0 Journal Article
%T Portal Hypertension and Myeloproliferative Neoplasms: A Relationship Revealed
%A Ahmet Burak Toros
%A Serkan Gokcay
%A Guven Cetin
%A Muhlis Cem Ar
%A Yesim Karagoz
%A Besir Kesici
%J ISRN Hematology
%D 2013
%R 10.1155/2013/673781
%X Background/Objectives. Patients with myeloproliferative neoplasms have a well-established increased risk of thrombosis. Many trials report identification of an underlying myeloproliferative neoplasm by investigation of the patients developing portal hypertensive esophagus and/or fundus variceal hemorrhage in the absence of any known etiology. This trial was designed to investigate the association between myeloproliferative neoplasms and portal hypertension and to detect the frequency of portal hypertension development in this subset of patients. Methodology. Twenty-nine patients previously diagnosed with polycythemia vera, essential thrombocytopenia, and primary myelofibrosis, who were under followup at the hematology outpatient clinic of our hospital, were included in the trial. Results. In our trial, we detected portal hypertension in 13.8% of the patients ( ), as a finding that was similar to those obtained in other studies performed to date. Conclusions. Considering the fact that diagnosis of myeloproliferative neoplasms usually takes a long time, treatment should be started (while, on the other hand, assessing the investigational and therapeutical choices for the complications) right after the bone marrow biopsy or cytogenetic studies required for establishing the final diagnosis have been performed. 1. Introduction The myeloproliferative neoplasms (MPN) include the Ph- (Philadelphia-) chromosome-positive chronic myeloid leukemia (CML), the Ph-chromosome-negative primary myelofibrosis (PMF), the polycythemia vera (PV), and the essential thrombocythemia (ET) [1¨C3]. Myeloproliferative neoplasms involve increased neoplastic proliferation in one or more series of the myeloid cells. In cases of polycythemia, overproduction of erythrocytes occurs. The increased erythrocyte mass leads to an increased blood viscosity. For PMF, extramedullary hematopoiesis (myeloid metaplasia) with the presence of myeloid, erythroid, and megakaryocytic series in locations other than the bone marrow such as the spleen, the liver, and the lymph nodes and fibrosis of various grades in the bone marrow suggest that the disease results from the hematopoietic stem cells [4¨C6]. Patients with PMF may develop severe portal hypertension, ascites, esophagus and fundus varices, intraluminal hemorrhage, and hepatic encephalopathy as a result of the massively increased splenoportal blood flow and the decreased hepatic vascular compliance or the hepatic venous thrombosis [7¨C9]. In essential thrombocythemia, an increase in the circulatory platelets occurs as a result of the continuous
%U http://www.hindawi.com/journals/isrn.hematology/2013/673781/