Hepatic Sinusoidal Obstruction Syndrome Secondary to Aplastic Anemia/Paroxysmal Nocturnal Hemoglobinuria Syndrome: A Rare Case.

ABSTRACT: Paroxysmal nocturnal hemoglobinuria (PNH) is associated with bone marrow failure disorders and may arise during the long-term follow-up of aplastic anemia (AA), which is named AA/PNH syndrome. Thrombosis is the most frequent clinical complication and is the main cause of mortality in PNH. However, thromboses tend to originate in hepatic and cerebral venous vessels, but rarely in the hepatic microvascular vein in PNH patients. Here, we report on a young man with hepatic sinusoidal obstruction syndrome (HSOS) secondary to AA/PNH syndrome. His main manifestations were hemolytic anemia, renal injury, ascites, hepatomegaly, and elevated liver enzymes. The diagnosis was confirmed by peripheral blood flow cytometry, enhanced computed tomography (CT), and liver biopsy. Initially, he received sympt

SUBMITTER: Liu X 

PROVIDER: S-EPMC12607371 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature