2014
DOI: 10.1111/imj.12609
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Vanishing bile duct syndrome and immunodeficiency preceding the diagnosis of Hodgkin lymphoma

Abstract: Vanishing bile duct syndrome (VBDS) in association with Hodgkin lymphoma (HL) is well described but not well understood. We report an unusual case of a 75-year-old patient presenting with biopsy-proven VBDS and immunodeficiency, without identifiable cause, which showed a waxing and waning course, culminating in the development of HL 18 months later. To our knowledge, this is the first adult case in which VBDS preceded the diagnosis of HL by such a long period.

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Cited by 7 publications
(2 citation statements)
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“…HL occurrence may be preceded by a VBDS [53,57] even by several months [53]. The period of time until HL appearance was 18 months in 75-year-old patient who also had a pre-existent unexplained immunodeficiency [57].…”
Section: Hodgkin's Lymphoma and Vanishing Bile Duct Syndromementioning
confidence: 99%
“…HL occurrence may be preceded by a VBDS [53,57] even by several months [53]. The period of time until HL appearance was 18 months in 75-year-old patient who also had a pre-existent unexplained immunodeficiency [57].…”
Section: Hodgkin's Lymphoma and Vanishing Bile Duct Syndromementioning
confidence: 99%
“…4 No specific biliary epithelial-associated autoantibody has been found in Hodgkin-associated VBDS. 5 There is no known association between EBV status and VBDS. Historically considered a poor prognostic factor, VBDS was associated with a 1-year overall survival of 43% in a series of 37 affected patients.…”
mentioning
confidence: 99%