[Primary hepatic diffuse large B-cell lymphoma developed in a patient with primary biliary cholangitis]

Area of Science:

• Hepatology and Oncology
• Immunology and Lymphoma Research

Background:

• Primary hepatic lymphoma (PHL) is a rare liver-confined lymphoproliferative disorder.
• Primary biliary cholangitis (PBC) and Sjögren's syndrome (SS) are autoimmune conditions that can be associated with lymphoproliferative disorders.
• The co-occurrence of PBC, SS, and PHL, particularly DLBCL-type, is exceptionally uncommon.

Purpose of the Study:

• To report a unique case of diffuse large B-cell lymphoma (DLBCL)-type PHL in a patient with a history of PBC and SS.
• To discuss the potential role of immune dysregulation in the pathogenesis of this rare condition.
• To highlight the clinical presentation, management, and outcome of this specific PHL subtype.

Main Methods:

• A case report of a 78-year-old woman presenting with epigastric pain and a solitary liver tumor.
• Diagnostic imaging included contrast-enhanced computed tomography (CT) to assess the liver lesion and rule out extrahepatic involvement.
• Surgical resection (laparoscopic left lateral segmentectomy) followed by histological examination of the tumor and affected liver parenchyma.
• Postoperative treatment with R-CHOP chemotherapy.

Main Results:

• Histological examination confirmed DLBCL in the liver, backgrounded by PBC-affected liver parenchyma.
• The patient had no evidence of extrahepatic lesions at presentation or on prior imaging.
• Following R-CHOP chemotherapy, the patient achieved a complete remission maintained for 4 years.

Conclusions:

• This case represents an extremely rare occurrence of DLBCL-type PHL associated with PBC and SS.
• Immune dysregulation stemming from PBC and secondary SS may be implicated in the development of DLBCL-type PHL.
• The successful management with R-CHOP chemotherapy suggests standard lymphoma treatment protocols are effective for this rare presentation.