Background: Vanishing bile duct syndrome (VBDS) in association with Hodgkin lymphoma (HL) is well described but not well understood. VBDS is an uncommon form of liver disease manifested by severe cholestasis and progressive liver failure. ­Specific pathogenesis is largely unknown but several hypothesis have been made, among which paraneoplastic manifestation, direct tumour cells infiltrations and drugs toxicity. Hypothermia is defined as a core body temperature less than 35 degrees C and it is often due to cold exposure and infections (especially caused by Gram-negative bacteria). The synchronous manifestation of both VBDS and severe hypothermia is a very rare condition and we will present a case of a male patient with a diagnosis of HL presenting both VBDS and hypothermia.

Patient Presentation: A 54-year-old male patient, with no history of significant co-morbidities, came to our attention suffering for cervical lymph nodes enlargement. A histologically proven diagnosis of HL lymphocytes-predominant variant was made. The patient presented also jaundice, and the following liver biopsy confirmed the diagnosis of VBDS. He was staged as IIIB (according to Ann Harbor classification) and was treated with ABVD chemotherapy. After the first cycle, the patient suffered for hypothermia, being his body temperature 33°C. He was treated with intravenous fluid replacement and cortisonics and his body temperature grew until the value of 37°C. A few day later, bilirubin value significantly decreased and he was able to sustain the second ABVD cycle, but unfortunately, he died after five days and his death was provoked by hemorrhagic pneumonia.

Discussion: VBDS is characterized by cholestasis and progressive destruction of the intrahepatic bile ducts (ductopenia). In the literature, various etiologies have been reported to cause ductopenia, with HL being listed as a rare example. How HL causes ductopenia remains ambiguous, and it seems to be related to a paraneoplastic phenomenon causing cytokine release from lymphoma cells. Hypothermia may be fatal if not promptly cured, and in particular, arrhythmia and cardiac arrest represent the major complications. It has been purposed as the causative agent an autonomic neuropathy, with hypothalamus involvement, on a paraneoplastic basis. This is the first case described in the literature of co-presentation of hypothermia and vanishing bile duct syndrome in a patient affected by HL, and as seen, the condition is associated with very poor prognosis and it needs of early detection and cure.