Vanishing Bile Duct Syndrome as a Manifestation of Paraneoplastic Syndrome in Hodgkin’s Lymphoma

T. P. Nekrasova; A. V. Berestova; P. A. Stribul; K. I. Miroslavin; M. S. Zharkova; A. V. Lapshin; A. V. Grigorenko; Yu. O. Shulpekova; V. T. Ivashkin

doi:10.22416/1382-4376-2025-35-4-104-112

Vanishing Bile Duct Syndrome as a Manifestation of Paraneoplastic Syndrome in Hodgkin’s Lymphoma

T. P. Nekrasova, A. V. Berestova, P. A. Stribul, K. I. Miroslavin, M. S. Zharkova, A. V. Lapshin, A. V. Grigorenko, Yu. O. Shulpekova, V. T. Ivashkin

https://doi.org/10.22416/1382-4376-2025-35-4-104-112

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Abstract

Aim: to present a clinical observation of paraneoplastic manifestations of Hodgkin’s lymphoma — liver damage in the development of vanishing bile duct syndrome.
Clinical case. Patient Sh., 18-years-old female, was admitted with complaints of yellowing of the skin and sclera, severe general weakness, and fever. Mechanical jaundice, viral hepatitis, Wilson — Konovalov disease, autoimmune hepatitis, and infections were excluded. A hypothesis was put forward about drug-induced hepatitis; some improvement was noted against the background of therapy with ursodeoxycholic acid and prednisolone, but fever and neutrophilic leukocytosis persisted. Physical examination and imaging revealed enlarged lymph nodes in various groups on both sides of the diaphragm, enlarged liver and spleen. PET-CT revealed active accumulation of 18F-fluorodeoxyglucose in the enlarged lymph nodes and bone marrow; lymphoproliferative disease was suspected. During follow- up, laboratory signs of cytolysis and cholestasis persisted, severe jaundice, decreased liver protein-synthetic function, and signs of portal hypertension were noted. A liver biopsy was performed, which revealed ductopenia in most portal tracts, without signs of inflammation (vanishing bile duct syndrome). The presence of splenomegaly, supra- and subdiaphragmatic lymphadenopathy contradicted the diagnosis of “drug-induced cholestasis”. A septic process was excluded. According to the examination, including histological examination of the enlarged lymph node, Hodgkin’s lymphoma, nodular sclerosis, with damage to the supra- and subdiaphragmatic lymph nodes, liver (“vanishing bile duct syndrome” — paraneoplastic reaction) and spleen, stage IIIB according to the Ann Arbor classification, were diagnosed. After polychemotherapy, the fever resolved, and laboratory parameters showed significant positive dynamics. Control PET-CT did not reveal foci of pathological accumulation of 18F-fluorodeoxyglucose. The patient continues to take ursodeoxycholic acid.
Conclusion. Liver involvement in Hodgkin’s lymphoma may manifest as vanishing bile duct syndrome, which is essentially a manifestation of paraneoplastic syndrome. Achieving complete remission and ursodeoxycholic acid therapy are considered to be the key to resolving ductopenia.

Keywords

vanishing bile duct syndrome, Hodgkin’s disease, paraneoplastic syndrome, cholestasis

About the Authors

T. P. Nekrasova

I.M. Sechenov First Moscow State Medical University (Sechenovskiy University)

Tatiyana P. Nekrasova — Cand. Sci. (Med.), Associate Professor at the Institute of Clinical Morphology and Digital
Pathology

119435, Moscow, Pogodinskaya str., 1, build. 1м

A. V. Berestova

I.M. Sechenov First Moscow State Medical University (Sechenovskiy University)
Russian Federation

Anna V. Berestova — Cand. Sci. (Med.), Associate Professor at the Institute of Clinical Morphology and Digital Pathology