Amarapurkar Pooja, Parekh Sunil, Amarapurkar Anjali, Amarapurkar Deepak
Department of Gastroenterology & Hepatology, Bombay Hospital and Medical Research Center, Hematology Laboratory, Mumbai, Maharashtra.
Dr Sunil Parekh's Lab & Consultant Histopathology, Mumbai, Maharashtra.
J Clin Exp Hepatol. 2012 Jun;2(2):188-90. doi: 10.1016/S0973-6883(12)60107-8. Epub 2012 Jul 21.
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).
骨髓增殖性疾病（MPD）是克隆性干细胞疾病，主要包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（IMF）。它们的特征是白细胞增多、血小板增多、红细胞增多、脾肿大和骨髓细胞增多。这也可能导致髓外造血。腹部表现已被认为是这些疾病的一个特征。脾肿大和肝肿大相当常见，而腹水则很少见。MPD主要影响肝循环系统。常见的肝脏表现为布加综合征（BCS）、门静脉血栓形成（PVT）和结节性再生性增生。MPD中出现的其他一些特征是由髓外造血、肝血流量增加和多次输血导致的继发性含铁血黄素沉着症引起的。高达7%的患者存在门静脉高压。我们报告一例接受经颈静脉肝内门体分流术（TIPS）治疗的髓外造血患者出现门静脉高压伴腹水的病例。
