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  • Case Study
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Cholestatic jaundice and bone lesions in an elderly woman

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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Figure 1: An endoscopic retrograde cholangiopancreatography image.
Figure 2: Results of an abdominal CT scan.
Figure 3: Hematoxylin and eosin staining of a sacral bone biopsy revealing a large mast-cell aggregate.
Figure 4: Liver biopsy and staining for peribiliary mast cells.

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Authors and Affiliations

Authors

Corresponding author

Correspondence to Mordechai Rabinovitz.

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Competing interests

The authors declare no competing financial interests.

Glossary

FOSINOPRIL

An angiotensin-converting enzyme inhibitor prescribed for cardiovascular diseases and hypertension

ICTERIC

Yellow discoloration of the skin and mucosal membranes, which is indicative of excess bilirubin

BRUSH CYTOLOGY

Tissue cell sampling for cytologic examination

CD117

(Tyrosine kinase Kit.) A cell-surface antigen involved in the signaling of tyrosine kinase, which is expressed on mast cell precursors

CD25

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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