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. 1987 Jul;28(7):903–906. doi: 10.1136/gut.28.7.903

Cholestasis and liver failure with lambda-AL amyloidosis.

F Konikoff 1, C Mor 1, S Stern 1, M Shaklai 1, J Halevy 1, E Theodor 1
PMCID: PMC1433083  PMID: 3115870

Abstract

Clinically significant liver involvement in systemic amyloidosis, especially with cholestasis, is rare. We report a case of primary amyloidosis with severe intrahepatic cholestasis leading to terminal liver failure. The present case is the first of its kind reported involving Lambda light chains only as the associated paraprotein. Conventional treatment and a therapeutic trial with dimethyl sulphoxide were unsuccessful.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BERO G. L. Amyloidosis: its clinical and pathologic manifestations, with a report of 12 cases. Ann Intern Med. 1957 May;46(5):931–955. doi: 10.7326/0003-4819-46-5-931. [DOI] [PubMed] [Google Scholar]
  2. Chopra S., Rubinow A., Koff R. S., Cohen A. S. Hepatic amyloidosis. A histopathologic analysis of primary (AL) and secondary (AA) forms. Am J Pathol. 1984 May;115(2):186–193. [PMC free article] [PubMed] [Google Scholar]
  3. Cox R. Amyloidosis of the liver causing jaundice. Postgrad Med J. 1982 Mar;58(677):192–193. doi: 10.1136/pgmj.58.677.192. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Finkelstein S. D., Fornasier V. L., Pruzanski W. Intrahepatic cholestasis with predominant pericentral deposition in systemic amyloidosis. Hum Pathol. 1981 May;12(5):470–472. doi: 10.1016/s0046-8177(81)80029-9. [DOI] [PubMed] [Google Scholar]
  5. Gertz M. A., Kyle R. A. Response of primary hepatic amyloidosis to melphalan and prednisone: a case report and review of the literature. Mayo Clin Proc. 1986 Mar;61(3):218–223. doi: 10.1016/s0025-6196(12)61853-9. [DOI] [PubMed] [Google Scholar]
  6. Kedar I., Greenwald M., Ravid M. Treatment of experimental murine amyloidosis with dimethyl sulfoxide. Eur J Clin Invest. 1977 Apr;7(2):149–150. doi: 10.1111/j.1365-2362.1977.tb01589.x. [DOI] [PubMed] [Google Scholar]
  7. Kyle R. A., Greipp P. R. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc. 1983 Oct;58(10):665–683. [PubMed] [Google Scholar]
  8. Kyle R. A., Greipp P. R., Garton J. P., Gertz M. A. Primary systemic amyloidosis. Comparison of melphalan/prednisone versus colchicine. Am J Med. 1985 Dec;79(6):708–716. doi: 10.1016/0002-9343(85)90521-2. [DOI] [PubMed] [Google Scholar]
  9. Kyle R. A., Greipp P. R. Primary systemic amyloidosis: comparison of melphalan and prednisone versus placebo. Blood. 1978 Oct;52(4):818–827. [PubMed] [Google Scholar]
  10. Kyle R. A., Pierre R. V., Bayrd E. D. Primary amyloidosis and acute leukemia associated with melphalan therapy. Blood. 1974 Sep;44(3):333–337. [PubMed] [Google Scholar]
  11. LEVINE R. A. Amyloid disease of the liver. Correlation of clinical, functional and morphologic features in forty-seven patients. Am J Med. 1962 Sep;33:349–357. doi: 10.1016/0002-9343(62)90231-0. [DOI] [PubMed] [Google Scholar]
  12. Levy M., Polliack A., Lender M., Eliakim M. The liver in amyloidosis. Digestion. 1974;10(1):40–51. doi: 10.1159/000197521. [DOI] [PubMed] [Google Scholar]
  13. Melato M., Manconi R., Magris D., Morassi P., Benussi D. G., Tiribelli C. Different morphologic aspects and clinical features in massive hepatic amyloidosis. Digestion. 1984;29(3):138–145. doi: 10.1159/000199023. [DOI] [PubMed] [Google Scholar]
  14. Melkebeke P., Vandepitte J., Hannon R., Fevery J. Huge hepatomegaly, jaundice and portal hypertension due to amyloidosis of the liver. Digestion. 1980;20(5):351–357. doi: 10.1159/000198459. [DOI] [PubMed] [Google Scholar]
  15. Mepham B. L., Frater W., Mitchell B. S. The use of proteolytic enzymes to improve immunoglobulin staining by the PAP technique. Histochem J. 1979 May;11(3):345–357. doi: 10.1007/BF01005033. [DOI] [PubMed] [Google Scholar]
  16. Pirovino M., Altorfer J., Maranta E., Hämmerli U. P., Schmid M. Ikterus vom Typ der intrahepatischen Cholestase bei Amyloidose der Leber. Z Gastroenterol. 1982 Jun;20(6):321–331. [PubMed] [Google Scholar]
  17. Ravid M., Shapira J., Lang R., Kedar I. Prolonged dimethylsulphoxide treatment in 13 patients with systemic amyloidosis. Ann Rheum Dis. 1982 Dec;41(6):587–592. doi: 10.1136/ard.41.6.587. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Rubinow A., Koff R. S., Cohen A. S. Severe intrahepatic cholestasis in primary amyloidosis: a report of four cases and a review of the literature. Am J Med. 1978 Jun;64(6):937–946. doi: 10.1016/0002-9343(78)90447-3. [DOI] [PubMed] [Google Scholar]
  19. Scheinberg M. A., Pernambuco J. C., Benson M. D. DMSO and colchicine therapy in amyloid disease. Ann Rheum Dis. 1984 Jun;43(3):421–423. doi: 10.1136/ard.43.3.421. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Taylor C. R. Immunoperoxidase techniques: practical and theoretical aspects. Arch Pathol Lab Med. 1978 Mar;102(3):113–121. [PubMed] [Google Scholar]

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