11 Apr 2021

2/25/21: Vanishing Bile Duct Syndrome

PGY2 Dana Toy presented a case of a 61 y/o male with history of HTN, HLD and Type 2 DM who presents with 1 month of progressively worsening jaundice and itching.  He initially noticed his skin and eyes appearing yellow followed by increased urinary frequency.  +Unintentional 15 pound weight loss over a few months, normal appetite, no nausea/vomiting/abdominal pain.  Patient was hospitalized for high sugars in Tijuana 1 month ago.

ROS: + urinary frequency, rest per HPI

Medications: Glargine 20U qhs, lispro sliding scale, Brazilian tea he uses to lower his sugars

FH: Breast cancer (mom), stomach cancer (cousin)

SH: not sexually active. Smoker (35 pack year), former drinker (drank a lot for 2 years in his 20s), former cocaine used in his 30s.  Denies IVDU

Vitals: T 36.9, HR 87, RR 18, BP 127/67,O2 sat 100% on RA

Physical Exam significant for jaundiced male, A&Ox4, no asterixis, +scleral icterus, normal cardiac/pulm/abdominal exam

CBC: WBC 4.4> Hb 10.5/Hct 31 <Plt 295 (MCV 97.4, RDW 16.6%)

CMP:

Na 120/K 4.2/Cl 89/HCO3 16/BUN 23/Cr 0.97<Glucose 156

ALP 1451>T.protein 4.8/Albumin 3/AST 348/ALT 522/Tbili 25.4/Dbili 19.2

Ca 9, Mg 1.8, Phos 3.4

Coags: PT 12.8, INR 0.98

UA with 100 protein, 250 glucose, large bilirubin, trace leukocytes (0-3 WBC), neg blood

Sosm 288 mOsm/kg, Uosm: 457, UNa: 48, UCr 84, FeNa 0.5%

Infectious workup (BCx, UCx, HIV, Hepatitis) all negative.

Autoimmune workup (ANA, ANCA, Actin, Mitochondrial Ab) all negative.

US Abdomen: Mild gallbladder wall thickening.  Normal liver echogenicity.  No intra- or extrahepatic bile duct dilatation.

CT A/P with contrast: Nonspecific enlarge periportal and peripancreatic lymph nodes.  No acute abnormalities within the pancreas.

PET w/ CT scan: multicompartmental hypermetabolic lymphadenpathy above and below the diaphragm.

Excisional LN biopsy: Classic Hodgkin lymphoma with numerous histiocytes

Liver biopsy: cholestatic hepatitis with focal interlobular bile duct atypia and focal duct loss.  Possibility of vanishing bile duct syndrome must be considered in this patient.

Treatment: Once Hodgkin lymphoma was diagnosed via biopsy, he was treated with cytarabine and dexamethasone in setting of hyperbilirubinemia.  Started on Brentuximab in the setting of HFrEF 40%.  On follow up, patient completed 9 cycles of Brentuximab and PET scan with no evidence of active lymphoproliferative disease.

Teaching points:

  1. Vanishing bile duct syndrome is a rare presentation of Hodgkin lymphoma.  It is an acquired disorder resulting in the disappearance of intrahepatic bile ducts and cholestasis.
  2. Progressive loss of intrahepatic bile ducts is associated with multiple etiologies including: neoplastic disorders, infectious, immunologic, toxins and drugs.
  3. Potential outcomes in patients with ductopenia:
    1. Irreversible bile duct loss leading to biliary cirrhosis
    2. Biliary epithelial regeneration and clinical recovery over months to years
  4. Liver damage is common in patients with Hodgkin lymphoma.  Prompt and appropriate treatment toward the underlying etiology can prevent intractable liver damage.
  5. Patients with compromised liver function can be managed initially with chemotherapy regimens that are safe in setting of liver dysfunction followed by standard regimens once liver function improves.

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