Chronic lymphocytic leukemia complicated by non-cirrhotic portal hypertension: an autopsy case report

Abstract

Portal hypertension typically occurs in cirrhosis but can rarely develop in the absence of hepatic fibrosis, particularly in hematologic malignancies such as chronic lymphocytic leukemia (CLL). We report a 70-year-old man with CLL who developed portal hypertension, presenting with massive splenomegaly, esophageal variceal rupture, and refractory ascites. He was initially diagnosed with alcohol-related cirrhosis based on a history of alcohol use and elevated liver stiffness on elastography. Chemotherapy had been discontinued due to cerebral hemorrhage and cytopenia. Ascites was refractory to and intolerant of diuretics and was worsened by renal dysfunction due to spontaneous bacterial peritonitis. After resolution of peritonitis, partial splenic artery embolization (PSE) was performed to relieve portal hypertension. Although ascites improved post-PSE, the patient developed a severe infection and died. Autopsy revealed no cirrhosis or hepatic fibrosis; instead, portal hypertension was attributed to CLL cell infiltration leading to splenomegaly and portal venous obstruction. This case underscores the need to consider hematologic malignancies as potential causes of non-cirrhotic portal hypertension. While PSE may offer symptomatic relief, the risk of fatal infection must be carefully weighed in patients with hematological malignancies. Histologic confirmation remains critical for accurate diagnosis in atypical presentations of portal hypertension.

Keywords: Autopsy; Chronic lymphocytic leukemia; Non-cirrhotic portal hypertension; Partial splenic embolization; Splenomegaly.