losartan-potassium and Budd-Chiari-Syndrome

Topics: Adult; Budd-Chiari Syndrome; Carcinoma, Hepatocellular; Erythropoiesis; Erythropoietin; Female; Humans; Liver Diseases; Liver Neoplasms; Male; Middle Aged; Polycythemia

Polycythaemia vera (PV) is a clonal disorder of bone marrow stem cells characterised by erythrocytosis. Diagnosis of PV requires exclusion of secondary causes of polycythaemia. It has been held that an elevated erythropoietin (Epo) level strongly indicates secondary erythrocytosis and excludes PV diagnosis, to the extent that the reduced serum Epo level is currently listed as a minor criterion in the WHO classification scheme for PV. However, patients with PV who co-present with Budd-Chiari syndrome have been documented with elevated serum Epo levels. For these patients, identification of the Janus kinase 2 (JAK2) V617F point mutation along with the transient nature of the Epo elevation provides certainty of PV diagnosis, as illustrated by the proband. In this case report, the patient's positive response to cytoreductive therapy (hydroxyurea 500 mg daily) and phlebotomy (750 mL over three phlebotomies) further supports validity of PV diagnosis with elevated Epo. The patient remains on rivaroxaban (Xarelto) for treatment of her portal vein thrombosis.

Topics: Ascites; Biomarkers; Budd-Chiari Syndrome; Diagnosis, Differential; Erythropoietin; Female; Humans; Middle Aged; Polycythemia Vera

It is widely accepted that an increased serum endogenous erythropoietin (Epo) level in a patient presenting with an elevated red cell mass makes a diagnosis of clonal polycythemia vera (PV) extremely unlikely. However, until the recent description of the constitutively activating V617F point mutation of the Janus 2 tyrosine kinase (JAK2)--a high-frequency molecular marker that is extremely specific for clonal chronic myeloproliferative disorders--distinction of PV from secondary erythrocytosis or other conditions has often been difficult. The purpose of this study was to use JAK2 V617F analysis to re-evaluate the validity of elevated Epo levels as a PV-exclusion criterion in patients with hepatic vein thrombosis [Budd-Chiari syndrome (BCS)].. We reviewed clinical data from 30 patients at our institution who presented with erythrocytosis and BCS. We isolated myeloid cells from fresh or archival bone marrow samples from four patients with BCS and an elevated serum Epo level, and analyzed them for the presence of the JAK2 V617F mutation.. All four samples were positive for JAK2 V617F, confirming the presence of a clonal hematopoietic disorder consistent with PV.. In the presence of BCS, elevated serum Epo levels do not exclude the diagnosis of PV.

Topics: Adult; Biomarkers; Budd-Chiari Syndrome; DNA Mutational Analysis; Erythropoietin; Female; Humans; Janus Kinase 2; Male; Middle Aged; Mutation, Missense; Polycythemia; Polycythemia Vera; Protein-Tyrosine Kinases; Proto-Oncogene Proteins

Topics: Adult; Budd-Chiari Syndrome; Dyspnea; Erythropoietin; Hepatomegaly; Humans; Hypertension, Pulmonary; Kidney Diseases, Cystic; Male; Polycythemia

Topics: Adult; Budd-Chiari Syndrome; Diagnosis, Differential; Erythropoietin; Female; Humans; Magnetic Resonance Spectroscopy; Polycythemia; Tomography, X-Ray Computed; Ultrasonography

We assessed the prevalence of overt and latent primary myeloproliferative disorders in hepatic vein thrombosis. Cultures of bone marrow or peripheral blood mononuclear cells were done in 20 patients with Budd-Chiari syndrome. Erythroid colony formation in the absence of erythropoietin, which is a reliable indicator for a primary myeloproliferative disorder, was seen in 16 patients in whom Budd-Chiari syndrome was due to hepatic vein thrombosis, including 13 women aged 18 to 45 years. Among these 16 patients, the conventional criteria for the diagnosis of a primary myeloproliferative disorder were met in only 2. Primary myeloproliferative disorder, often without peripheral blood changes, is a major cause of hepatic vein thrombosis in young women.

Topics: Adolescent; Adult; Budd-Chiari Syndrome; Colony-Forming Units Assay; Contraceptives, Oral; Erythrocyte Volume; Erythropoiesis; Erythropoietin; Female; Hematopoietic Stem Cells; Humans; Male; Middle Aged; Myeloproliferative Disorders; Polycythemia Vera; Prospective Studies