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Case Studies | Medical Science | India | Volume 12 Issue 9, September 2023 | Rating: 4.8 / 10
Unraveling the Connection: PNH Induced Budd-Chiari Syndrome - A Case Study and Early Detection Emphasis
Prachi Parikh [2] | Darsh Patel [2] | Rachit Shah | Parth Patel [3]
Abstract: PNH is acquired disorder of hematopoietic stem cell which is characterized by acquired intravascular hemolytic anemia, bone marrow failure with pancytopenia and high incidence of life threatening thrombosis. A serious complication of PNH is hepatic venous thrombosis leading to Budd Chiari syndrome. Budd-Chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. The obstruction may be thrombotic or non-thrombotic anywhere along the venous course from the hepatic venules to junction of the inferior vena cava (IVC) to the right atrium. Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. We present a rare case of PNH in a 23 year old man clinically presenting with Budd Chiari syndrome that on detailed investigations actually turned out to be complication of PNH. This article emphasizes on early detection of PNH to prevent life threatening complications like Budd Chiari syndrome.
Keywords: PNH, Budd Chiari Syndrome, Hepatic Venous Thrombosis
Edition: Volume 12 Issue 9, September 2023,
Pages: 37 - 40