Progression of morphological changes after transplantation of a liver with heterozygous α -1 antitrypsin deficiency

Summary Inadvertent transplantation of an α -1 antitrypsin–deficient liver into an adult man provided a unique opportunity to follow the natural history of morphological changes in serial liver biopsies. After doing well initially, the patient developed liver function test abnormalities 6 years post...

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Bibliographic Details
Published in:Human pathology 2012-05, Vol.43 (5), p.753-756
Main Authors: Lee, Sun-Mi, MD, Speeg, K. Vincent, MD, PhD, Pollack, Marilyn S., PhD, Sharkey, Francis E., MD
Format: Article
Language:English
Subjects:
Adult
alpha 1-Antitrypsin Deficiency - pathology
Biological and medical sciences
Chronic hepatitis
Disease Progression
Gastroenterology. Liver. Pancreas. Abdomen
Hepatopathology
Humans
Investigative techniques, diagnostic techniques (general aspects)
Liver - pathology
Liver Diseases - pathology
Liver Function Tests
Liver Transplantation
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Other diseases. Semiology
Pathology
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
α-1 antitrypsin deficiency
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Summary:Summary Inadvertent transplantation of an α -1 antitrypsin–deficient liver into an adult man provided a unique opportunity to follow the natural history of morphological changes in serial liver biopsies. After doing well initially, the patient developed liver function test abnormalities 6 years posttransplant, but biopsies at that time and 2 years later revealed only chronic hepatitis with no specific features. It was only upon repeat biopsy 10 years posttransplant that characteristic cytoplasmic inclusions appeared. Genotypic and phenotypic testing of pretransplant and posttransplant specimens confirmed α -1 antitrypsin deficiency in the transplanted liver. Serologic tests for viral hepatitis and autoimmune disease were negative throughout the pretransplant and posttransplant period. The case suggests that patients with chronic hepatitis of unknown etiology should be tested for the possibility of α -1 antitrypsin deficiency and illustrates the prolonged course that may precede the development of typical cytoplasmic inclusions in the liver.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2011.08.001