title: Portal hypertension and ascites in extramedullary hematopoiesis.
abstract: Myeloproliferative diseases (MPD) are clonal stem cell disorders which mainly include polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (IMF). They are characterized by leucocytosis, thrombocytosis, erythrocytosis, splenomegaly, and bone marrow hypercellularity. This might also result in extramedullary hematopoiesis. Abdominal manifestation has been recognized as a feature of these disorders. Splenomegaly and hepatomegaly are fairly common as opposed to ascites which is rare. The MPDs mainly affect the hepatic circulatory systems. The common hepatic manifestations are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT), and nodular regenerative hyperplasia. A few other features seen in MPDs are caused by extramedullary hematopoiesis, increased hepatic blood flow, and secondary hemosiderosis from multiple blood transfusions. Portal hypertension is found in up to 7% of patients. We report a case of portal hypertension with ascites in a patient with extramedullary hematopoiesis treated with transjugular intrahepatic portocaval shunt (TIPS).
authors.affiliation: Department of Gastroenterology & Hepatology, Bombay Hospital and Medical Research Center, Hematology Laboratory, Mumbai, Maharashtra.
authors.forename: Pooja
authors.initials: P
authors.lastname: Amarapurkar
authors.affiliation: Dr Sunil Parekh's Lab & Consultant Histopathology, Mumbai, Maharashtra.
authors.forename: Sunil
authors.initials: S
authors.lastname: Parekh
authors.affiliation: SRL & Dr Avinash Phadke Laboratory, Mumbai, Maharashtra.
authors.forename: Anjali
authors.initials: A
authors.lastname: Amarapurkar
authors.affiliation: Department of Gastroenterology & Hepatology, Bombay Hospital and Medical Research Center, Hematology Laboratory, Mumbai, Maharashtra.
authors.forename: Deepak
authors.initials: D
authors.lastname: Amarapurkar
keywords: BCS, Budd-Chiari syndrome; ET, essential thrombocythemia; Extramedullary hematopoiesis; IMF, idiopathic myelofibrosis; IVC, inferior vena cava; MPD, myeloproliferative diseases; PV, polycythemia vera; PVT, portal vein thrombosis; SAAG, serum ascitic fluid albumin gradient; TIPS; TIPS, transjugular intrahepatic portocaval shunt; myeloproliferative diseases