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Related Experiment Videos

Hepatic involvement in systemic mast cell disease.

L T Yam, C H Chan, C Y Li

    The American Journal of Medicine
    |May 1, 1986
    PubMed
    Summary
    This summary is machine-generated.

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    Systemic mast cell disease can cause liver fibrosis and inflammation. While hepatic function tests are usually normal, alkaline phosphatase is elevated in all patients, but liver changes do not impact prognosis.

    Area of Science:

    • Hepatology
    • Gastroenterology
    • Pathology

    Background:

    • Systemic mast cell disease (SMCD) is a rare multisystem disorder.
    • Hepatic involvement is common in SMCD, often presenting as hepatomegaly.

    Purpose of the Study:

    • To define hepatic changes in SMCD.
    • To correlate liver histology with clinical findings.

    Main Methods:

    • Histological examination of liver biopsy specimens from 13 SMCD patients.
    • Special staining techniques (Giemsa, toluidine blue, chloroacetate esterase) for mast cells.
    • Correlation of histological findings with clinical and biochemical data.

    Main Results:

    • All patients showed liver fibrosis and chronic inflammatory infiltration in portal areas.

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  • Hepatic sinusoids were not significantly involved.
  • Elevated alkaline phosphatase was observed in all patients; other liver function tests were mostly normal.
  • Histological severity did not correlate with liver size or blood biochemistry.
  • Abnormal serum biochemistry was linked to dehydration and malnutrition.

    • Conclusions:

    • Histological diagnosis of SMCD in liver biopsies can be challenging with standard stains but improved with plastic embedding and special stains.
    • Hepatic involvement in SMCD appears to have minimal direct impact on prognosis.
    • Prognosis is primarily determined by systemic mast cell burden in other organs.