Hodgkin-lymphomához társult eltűnőepeút-szindróma

2021 ◽  
Vol 162 (22) ◽  
pp. 884-888
Author(s):  
Szandra Boldizsár ◽  
János Rottek ◽  
Tamás Schneider ◽  
Fatima Varga ◽  
Erika Szaleczky

Összefoglaló. Az eltűnőepeút-szindróma ritka, rossz prognózisú kórkép. Az epeutak progresszív destrukciójával, az intrahepaticus epeutak eltűnésével jár, epepangáshoz, biliaris cirrhosishoz, végül májelégtelenséghez vezet. A háttérben álló kiváltó okok között infekciók, ischaemia, gyógyszermellékhatások, illetve daganatos megbetegedések szerepelhetnek. A malignitások közül a leggyakrabban a Hodgkin-lymphomához társult formájával találkozhatunk. Cikkünkben egy fiatal, Hodgkin-lymphomás betegünk esetét szeretnénk bemutatni, akinél az icterus hátterében eltűnőepeút-szindróma igazolódott, melyet egyéb okok kizárását követően szövettani mintavétellel igazoltunk. A két ciklus ABVD-protokoll szerinti kezelést követő PET/CT az alapbetegség tekintetében komplett metabolikus remissziót igazolt. A klinikai javuláshoz azonban hosszú hónapokra volt szükség. Végül az epeúteltűnés esetünkben reverzibilis folyamatnak bizonyult, az alapbetegség tekintetében a komplett metabolikus remisszió elérésével az epeút-károsodás megállítható volt. Orv Hetil. 2021; 162(22): 884–888. Summary. destruction and loss of the intrahepatic bile ducts leading to cholestatis, biliar chirrosis and finally liver failure. It has been described in different pathologic conditions including infections, ischemia, adverse drug reactions and malignancies. The Hodgkin’s lymphoma-associated type occurs most frequently among the forms of the disease of malignant origin. In this report, we introduce the case of a 32-year-old male patient with Hodgkin’s lymphoma, diagnosed with vanishing bile duct syndrome upon liver biopsy as a root cause behind his icterus. The PET/CT has proven complete metabolic remission after 2 cycles of ABVD chemoterapy. Clinical improvement, however, occurred only after several months. Finally the loss of bile ducts proved to be a reversible process, the complete metabolic remission of Hodgkin’s lymphoma resulted in the regeneration of the bile ducts. Orv Hetil. 2021; 162(22): 884–888.

2021 ◽  
Vol 8 (9) ◽  
Author(s):  
Keramidas V ◽  
◽  
Tastanis C ◽  
Tsirogianni K ◽  
Hytiroglou P ◽  
...  

Vanishing Bile Duct Syndrome (VBDS) is a rare, acquired disorder, characterized by progressive destruction and loss of intrahepatic bile ducts. The main clinical manifestations are jaundice and pruritus, caused by intralobular cholestasis. Although the pathogenic mechanism is poorly understood, VBDS has been associated with numerous etiologies such as medications, malignancies, infections and autoimmune diseases. This syndrome can appear as a paraneoplastic phenomenon in patients with Hodgkin’s Lymphoma (HL). Diagnosis is based on clinical evaluation and confirmed via liver biopsy, while treating the underlying cause is the main therapeutic target. If bile duct regeneration does not occur, possible outcomes include cirrhosis, hepatic failure and death, with liver transplantation being the only curative option. In this paper, we describe a case of HL-related VBDS in a 31-year-old female patient, who presented with jaundice, pruritus and cervical lymphadenopathy. The stage of HL was determined as IIA and a liver biopsy was performed, which confirmed the degeneration of bile ducts. The patient was treated with the ABVD regimen and dexamethasone. Follow-up tests were normal and supported the full remission hypothesis. We conducted an analytical literature review and collected the available data from 38 confirmed cases, regarding the epidemiology, viral infections, clinical findings, therapeutic options and outcome.


2019 ◽  
Vol 12 (2) ◽  
pp. bcr-2018-227676 ◽  
Author(s):  
Min Fong ◽  
Stephen Boyle ◽  
Naadir Gutta

We present a novel treatment protocol that was successful in the management of Hodgkin’s-associated vanishing bile duct syndrome, a rare but serious complication of Hodgkin’s lymphoma. We believe that publication of this treatment protocol and the rationale for its development will be of interest to anyone faced with treating this challenging condition.


2016 ◽  
Vol 50 (8) ◽  
pp. 688 ◽  
Author(s):  
Mena Bakhit ◽  
Thomas R. McCarty ◽  
Sunhee Park ◽  
Basile Njei ◽  
Margaret Cho ◽  
...  

2018 ◽  
pp. 37-40
Author(s):  
Erdem Sunger ◽  
Elif Yorulmaz ◽  
Ahmet Engin Atay ◽  
Burcak Karadeniz ◽  
Husniye Esra Pasaoglu ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document