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%)
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.
- 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.
- Progressive loss of intrahepatic bile ducts is associated with multiple etiologies including: neoplastic disorders, infectious, immunologic, toxins and drugs.
- Potential outcomes in patients with ductopenia:
- Irreversible bile duct loss leading to biliary cirrhosis
- Biliary epithelial regeneration and clinical recovery over months to years
- Liver damage is common in patients with Hodgkin lymphoma. Prompt and appropriate treatment toward the underlying etiology can prevent intractable liver damage.
- 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.