Hiraide Akiri, Ojiro Keisuke, Matsumoto Kimihiro, Katayama Tadashi, Nakamura Kenji, Kishikawa Hiroshi, Sasaki Aya, Nakamoto Nobuhiro, Nishida Jiro
Department of Gastroenterology and Hepatology, Tokyo Dental College Ichikawa General Hospital, 5-11-13 Sugano, Ichikawa-shi, Chiba, 272-8513, Japan.
Division of Gastroenterology, Department of Internal Medicine, Keio University, 35 Shinanomachi , Shinjuku-ku, Tokyo, 160-8582, Japan.
Clin J Gastroenterol. 2025 Jul 16. doi: 10.1007/s12328-025-02184-1.
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.
门静脉高压通常发生在肝硬化患者中，但在没有肝纤维化的情况下也很少会出现，尤其是在慢性淋巴细胞白血病（CLL）等血液系统恶性肿瘤患者中。我们报告了一名70岁的CLL男性患者，他出现了门静脉高压，表现为巨大脾肿大、食管静脉曲张破裂和难治性腹水。他最初因有饮酒史且弹性成像显示肝脏硬度升高而被诊断为酒精性肝硬化。由于脑出血和血细胞减少，化疗已停止。腹水对利尿剂难治且不耐受，因自发性细菌性腹膜炎导致的肾功能不全而加重。腹膜炎消退后，进行了部分脾动脉栓塞术（PSE）以缓解门静脉高压。尽管PSE术后腹水有所改善，但患者发生了严重感染并死亡。尸检显示没有肝硬化或肝纤维化；相反，门静脉高压归因于CLL细胞浸润导致脾肿大和门静脉阻塞。该病例强调了需要将血液系统恶性肿瘤视为非肝硬化性门静脉高压的潜在原因。虽然PSE可能会缓解症状，但对于血液系统恶性肿瘤患者，必须仔细权衡致命感染的风险。组织学确诊对于门静脉高压非典型表现的准确诊断仍然至关重要。
