Hepatic Amyloidosis (AL): The Natural History in 80 Patients

  • Morie A. Gertz
  • Robert A. Kyle


We followed 80 patients with liver biopsy-proven primary amyloidosis (AL) to study its natural history. Liver biopsy does not carry a major risk of bleeding or rupture. Although the liver dominated the clinical presentation, most patients had evidence of systemic involvement. Overall, 77 percent had an associated nephrotic syndrome, congestive heart failure, or neuropathy. The history and physical examination was not helpful in differentiating hepatic amyloidosis from other forms of liver disease. Laboratory studies that suggested the diagnosis were proteinuria (88 percent) and either a monoclonal band or hypogammaglobulinemia on serum protein electrophoresis (64 percent) or hyposplenism on the peripheral blood smear (51 percent). The value of protein electrophoresis was enhanced by immuno-electrophoresis of the serum and urine. When this was done, a monoclonal protein was detected in 76 percent. Kappa light chains were not associated with giant hepatomegaly. Hepatomegaly occurred in the absence of abnormal liver function tests (32 percent). Although the alkaline phospha-tase at diagnosis had no impact on survival, a two-fold elevation in SGOT or bilirubin predicted a median survival of four months. Myeloma was diagnosed in 12 but had no effect on the clinical course. The commonest coagulation abnormality was prolongation of the thrombin time. No coagulation parameter predicted bleeding risk. The median survival was nine months with projected five-and ten-year survivals of 13 percent and 1 percent. Five patients had indolent disease surviving 60+ months. These survivors had no distinguishing features that permitted their recognition prospec-tively. There was no impact of treatment on the course of the disease.


Liver Biopsy Nephrotic Syndrome Carpal Tunnel Syndrome Thrombin Time Peripheral Blood Smear 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    W. M. Butler, and P.E. Baldwin, South Med J 77:648–651 (1984).CrossRefGoogle Scholar
  2. 2.
    A. S. Cohen, and M. Skinner, in: “Diseases of the liver,” L. Schiff, and E. R. Schiff eds., JB Lippincott Company, Philadelphia (1982).Google Scholar
  3. 3.
    D. C. Dahlin, Proc Staff Meet Mayo Clin 24:637–648 (1949).Google Scholar
  4. 4.
    S. D. Finkelstein, V. L. Fornasier, and W. Pruzanski, Hum Pathol 12:470–472 (1981).CrossRefGoogle Scholar
  5. 5.
    M. A. Gertz, and R. A. Kyle, Mayo Clin Proc 61:218–223 (1986).CrossRefGoogle Scholar
  6. 6.
    M. A. Gertz, R. A. Kyle, and P. R. Greipp, Ann Intern Med 98:475–477 (1983).CrossRefGoogle Scholar
  7. 7.
    P. R. Greipp, R. A. Kyle, and E. J. Bowie, Am J Hematol 11:443–450 (1981).CrossRefGoogle Scholar
  8. 8.
    R. A. Kyle, and P. R. Greipp, Mayo Clin Proc 58:665–683 (1983).Google Scholar
  9. 9.
    R. A. Kyle, P. R. Greipp, J. P. Garton, and M. A. Gertz, Am J Med 79:708–716 (1985).CrossRefGoogle Scholar
  10. 10.
    R. A. Kyle, P. R. Greipp, and W. M. O’Fallon, Blood 68:220–224 (1986).Google Scholar
  11. 11.
    R. A. Kyle, R. V. Pierre, and E. D. Bayrd, Blood 44:333–337 (1974).Google Scholar
  12. 12.
    R. A. Levine, Am J Med 33:349–357 (1962).CrossRefGoogle Scholar
  13. 13.
    M. Levy, C. H. Fryd, and M. Eliakim, Gastroenterology 61:234–238 (1971).Google Scholar
  14. 14.
    M. Levy, A. Polliack, M. Lender, and M. Eliakim, Digestion 10:40–51 (1974).CrossRefGoogle Scholar
  15. 15.
    A. Polliack, and C. Hershko, Isr J Med Sci 8:57–60 (1972).Google Scholar
  16. 16.
    M. Pras, B. Frangione, E. C. Franklin, and J. Gafni. Isr J. Med Sci 18:866–869 (1982).Google Scholar
  17. 17.
    A. Rubinow, R. S. Koff, and A. S. Cohen, Am J Med 64:937–946 (1978).CrossRefGoogle Scholar
  18. 18.
    S. H. Salzman, and G. Burke, Am J Gastroenterol 47:221–230 (1967).Google Scholar
  19. 19.
    A. Solomon, B. Frangione, and E. C. Franklin, J Clin Invest 70:453–460 (1982).CrossRefGoogle Scholar
  20. 20.
    D. M. Spain, and R. L. Riley, Am J Clin Pathol 14:284–288 (1944).Google Scholar
  21. 21.
    M. H. Stauffer, J. B. Gross, W. T. Foulk, and D. C. Dahlin, Gastroenterology 41:92–96 (1961).Google Scholar
  22. 22.
    R. A. Yood, M. Skinner, A. Rubinow, L. Talarico, and A. S. Cohen, JAMA 249:1322–1324 (1983).CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Morie A. Gertz
    • 1
  • Robert A. Kyle
    • 1
  1. 1.Division of Hematology, Dysproteinemia Clinic and Department of Laboratory MedicineMayo Clinic and Mayo FoundationRochesterUSA

Personalised recommendations