Abstract
Background A 74-year-old Caucasian female presented with jaundice, clay-colored stools, diarrhea, and fatigue of 3 months' duration, accompanied by a weight loss of 6.8 kg. Physical examination demonstrated mild hepatomegaly. Initial blood work revealed abnormal liver biochemistries with a cholestatic pattern. An abdominal CT scan showed intrahepatic bile-duct dilatation without masses but with multiple lytic and blastic bone lesions. A sacral bone biopsy established the diagnosis.
Investigations Endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, CT scan, bone biopsy and liver biopsy.
Diagnosis Systemic mastocytosis affecting the biliary system resulting in a primary-sclerosing-cholangitis-like picture combined with diffuse blastic and lytic bone lesions.
Management Biliary stenting, histamine 1- and 2-receptor blockers, and chemotherapy (cladribine).
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Glossary
- FOSINOPRIL
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An angiotensin-converting enzyme inhibitor prescribed for cardiovascular diseases and hypertension
- ICTERIC
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Yellow discoloration of the skin and mucosal membranes, which is indicative of excess bilirubin
- BRUSH CYTOLOGY
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Tissue cell sampling for cytologic examination
- CD117
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(Tyrosine kinase Kit.) A cell-surface antigen involved in the signaling of tyrosine kinase, which is expressed on mast cell precursors
- CD25
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A cell-surface antigen, acting as an interleukin-2 receptor, which is aberrantly expressed on mast cells
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Papachristou, G., Demetris, A., Craig, F. et al. Cholestatic jaundice and bone lesions in an elderly woman. Nat Rev Gastroenterol Hepatol 1, 53–57 (2004). https://doi.org/10.1038/ncpgasthep0026
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DOI: https://doi.org/10.1038/ncpgasthep0026
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