Lohana Ajeet Kumar, Tariq Muhammad Ali, Abid Shahab
Aga Khan University Hospital
Tower Health
Primary hepatic lymphoma (PHL) is a rare form of non-Hodgkin lymphoma (NHL) that primarily involves the liver, as opposed to a predominant lymph nodal or splenic involvement in other subtypes of NHL. The liver is the major reticuloendothelial organ, and hepatic involvement secondary to systemic NHL is common, such that 40% of patients with NHL have liver involvement. The majority of the patients with PHL report vague symptoms such as nausea, vomiting, upper abdominal pain or discomfort, and approximately one-third report constitutional symptoms including fever, myalgias, and weight loss. However, due to the low incidence with characteristically vague symptoms at the outset, patients with PHL often undergo extensive investigations before reaching up a definitive diagnosis. Diagnosis of PHL depends on a liver biopsy that should be compatible with the lymphoma and the absence of extrahepatic lymphoproliferative involvement. Primary hepatic lymphoma may often confound with other space-occupying liver lesions, namely hepatocellular carcinoma, hepatic adenoma, focal hyperplasia of the liver, and hepatic hemangioma. At times a hepatologist must address the rare possibility of PHL besides the common notably, hepatocellular carcinoma while approaching the space-occupying lesions of the liver.
原发性肝淋巴瘤(PHL)是一种罕见的非霍奇金淋巴瘤(NHL),主要累及肝脏,这与其他NHL亚型主要累及淋巴结或脾脏不同。肝脏是主要的网状内皮器官,系统性NHL继发肝脏受累很常见,40%的NHL患者有肝脏受累。大多数PHL患者报告有恶心、呕吐、上腹部疼痛或不适等模糊症状,约三分之一的患者报告有包括发热、肌痛和体重减轻在内的全身症状。然而,由于PHL发病率低且起病时症状典型模糊,PHL患者在确诊前往往要接受广泛的检查。PHL的诊断依赖于肝脏活检,活检结果应与淋巴瘤相符且无肝外淋巴增殖性受累。原发性肝淋巴瘤常与其他肝脏占位性病变混淆,即肝细胞癌、肝腺瘤、肝脏局灶性增生和肝血管瘤。有时,肝病学家在处理肝脏占位性病变时,除了常见的尤其是肝细胞癌外,还必须考虑到PHL这种罕见的可能性。