A case of recurrent follicular cholangitis leading to decompensated cirrhosis after left-sided hepatectomy.

Follicular cholangitis (FC) is a rare non-neoplastic biliary tract disease first reported in 2003. A 74-year-old woman underwent extended left hepatectomy with a diagnosis of intrahepatic cholangiocarcinoma. Histopathological examination of the surgical specimen demonstrated no malignant findings, and lymphocytic infiltration with lymphoid follicles was observed within the bile duct wall. Along with immunohistochemical findings, the patient was diagnosed with FC. More than 3 years after surgery, the patient exhibited elevated hepatobiliary enzymes and total bilirubin. Endoscopic retrograde cholangiography revealed stricture and dilation from the extrahepatic bile duct to the right intrahepatic bile duct. Histopathological findings uncovered lymphocytic infiltration without malignant results. It was concluded that bile duct stricture due to FC had newly developed in her remnant liver. Subsequently, the patient developed hypoalbuminemia, and abdominal computed tomography revealed atrophy of the remnant liver and ascites accumulation. Esophagogastroduodenoscopy exposed the development of esophageal varices, which were not observed preoperatively. The patient was diagnosed with decompensated liver cirrhosis accompanied by portal hypertension. This case strongly suggests that long-term follow-up after surgery may be required for patients with FC for screening of potential new bile duct stricture and progression to liver cirrhosis due to cholestasis.

Primary Hepatic Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders After Methotrexate Therapy.

Prior reports described cases of lymphoproliferative diseases occurring after methotrexate (MTX) administration, which are called methotrexate-associated lymphoproliferative disorders (MTX-LPDs). It has become clear that these lymphoproliferative diseases also occur following treatment with other immunosuppressive drugs, and they have been termed as other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs). In most of these cases, the duration of immunosuppressive drugs is very long, on the order of years. In the present study, we evaluated the development of lymphoproliferative disease despite the short duration of immunosuppressive treatment and determined the tumor doubling time. A 71-year-old woman was diagnosed with adult-onset Still's disease. The patient was administered prednisone 30 mg per day starting on February 25, 2022 and MTX 6 mg per week starting 2 weeks later. Because she was a hepatitis B virus (HBV) carrier, nucleic acid analog therapy was also started to prevent HBV activation. Eight weeks later, biweekly tocilizumab was started. After 5 months of MTX administration, a solitary liver tumor measuring 37 × 32 mm2 was detected. Three months later, repeat computed tomography revealed that the liver tumor had grown rapidly to 7 cm in diameter. We considered the possibility of OIIA-LPDs and stopped MTX therapy. Biopsy specimens of the liver tumor exhibited lymphocyte proliferation, which was consistent with OIIA-LPDs. The doubling time for tumor growth was 33 days. Despite withdrawing MTX for 6 weeks, the tumor continued to grow, and thus, the patient was referred to the hematology unit. In previously reported cases of MTX-LPDs of hepatic origin, the average duration of MTX administration was 7.3 (2 - 13) years. This report describes a primary hepatic OIIA-LPDs-associated tumor that rapidly increased in size after an extremely short period of MTX administration.