Abstract
The liver is a major site of amyloid deposition. The spectrum of histopathologic changes in the liver was studied in 38 patients with systemic amyloidosis (25 with primary or myeloma-associated amyloidosis [AL] and 13 with secondary, reactive [AA] amyloidosis). Overall architectural distortion, alterations of portal triads, as well as predilection for topographic deposition in the parenchyma and/or blood vessel walls were noted. Significant histopathologic differences in AL or AA amyloid liver involvement included 1) portal fibrosis, seen in 7 of 25 (28%) AL patients and 8 of 13 (62%) AA patients (P = 0.05), 2) parenchymal amyloid deposition in 25 of 25 (100%) AL amyloid and 10 of 13 (77%) AA amyloid patients (P = 0.04), and 3) vascular amyloid deposition found in 17 of 25 (68%) with AL amyloid and 13 of 13 (100%) patients with AA amyloid (P = 0.02). These data vary from the widely held concept that deposition of amyloid is predominantly vascular in the AL form and parenchymal in amyloid AA. Clearly, however, in individual cases significant overlap occurred, and characterization of amyloid types based on morphologic distribution of amyloid deposits may be possible in only a minority of cases. In most cases, differentiation of amyloid AL and amyloid AA forms requires clinical, histochemical, immunochemical, and sometimes more elaborate laboratory amino acid sequence studies for accurate identification.
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- Brandt K., Cathcart E. S., Cohen A. S. A clinical analysis of the course and prognosis of forty-two patients with amyloidosis. Am J Med. 1968 Jun;44(6):955–969. doi: 10.1016/0002-9343(68)90095-8. [DOI] [PubMed] [Google Scholar]
- Carson F. L., Kingsley W. B. Nonamyloid green birefringence following Congo red staining. Arch Pathol Lab Med. 1980 Jun;104(6):333–335. [PubMed] [Google Scholar]
- Celli B. R., Rubinow A., Cohen A. S., Brody J. S. Patterns of pulmonary involvement in systemic amyloidosis. Chest. 1978 Nov;74(5):543–547. doi: 10.1378/chest.74.5.543. [DOI] [PubMed] [Google Scholar]
- Cohen A. S. Amyloidosis. N Engl J Med. 1967 Sep 7;277(10):522–contd. doi: 10.1056/NEJM196709072771006. [DOI] [PubMed] [Google Scholar]
- Cohen A. S., Shirahama T., Sipe J. D., Skinner M. Amyloid proteins, precursors, mediator, and enhancer. Lab Invest. 1983 Jan;48(1):1–4. [PubMed] [Google Scholar]
- French S. W., Schloss G. T., Stillman A. E. Unusual amyloid bodies in human liver. Am J Clin Pathol. 1981 Mar;75(3):400–402. doi: 10.1093/ajcp/75.3.400. [DOI] [PubMed] [Google Scholar]
- Glenner G. G. Amyloid deposits and amyloidosis. The beta-fibrilloses (first of two parts). N Engl J Med. 1980 Jun 5;302(23):1283–1292. doi: 10.1056/NEJM198006053022305. [DOI] [PubMed] [Google Scholar]
- Glenner G. G., Terry W., Harada M., Isersky C., Page D. Amyloid fibril proteins: proof of homology with immunoglobulin light chains by sequence analyses. Science. 1971 Jun 11;172(3988):1150–1151. doi: 10.1126/science.172.3988.1150. [DOI] [PubMed] [Google Scholar]
- Gregg J. A., Herskovic T., Bartholomew L. G. Ascites in systemic amyloidosis. Arch Intern Med. 1965 Oct;116(4):605–610. [PubMed] [Google Scholar]
- Hurd W. W., Katholi R. E. Acquired functional asplenia. Association with spontaneous rupture of the spleen and fatal spontaneous rupture of the liver in amyloidosis. Arch Intern Med. 1980 Jun;140(6):844–845. doi: 10.1001/archinte.140.6.844. [DOI] [PubMed] [Google Scholar]
- Johannessen J. V., Gould V. E., Jao W. The fine structure of human thyroid cancer. Hum Pathol. 1978 Jul;9(4):385–400. doi: 10.1016/s0046-8177(78)80025-2. [DOI] [PubMed] [Google Scholar]
- KAPP J. P. HEPATIC AMYLOIDOSIS WITH PORTAL HYPERTENSION. JAMA. 1965 Feb 8;191:497–499. doi: 10.1001/jama.1965.03080060071020. [DOI] [PubMed] [Google Scholar]
- Kanel G. C., Uchida T., Peters R. L. Globular hepatic amyloid--an unusual morphologic presentation. Hepatology. 1981 Nov-Dec;1(6):647–652. doi: 10.1002/hep.1840010614. [DOI] [PubMed] [Google Scholar]
- 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]
- Levin M., Pras M., Franklin E. C. Immunologic studies of the major nonimmunoglobulin protein of amyloid. I. Identification and partial characterization of a related serum component. J Exp Med. 1973 Aug 1;138(2):373–380. doi: 10.1084/jem.138.2.373. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 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]
- Livni N., Behar A. J., Lafair J. S. Unusual amyloid bodies in human liver. Ultrastructural and freeze-etching studies. Isr J Med Sci. 1977 Dec;13(12):1163–1170. [PubMed] [Google Scholar]
- Rubinow A., Cohen A. S. Skin involvement in generalized amyloidosis. A study of clinically involved and uninvolved skin in 50 patients with primary and secondary amyloidosis. Ann Intern Med. 1978 Jun;88(6):781–785. doi: 10.7326/0003-4819-88-6-781. [DOI] [PubMed] [Google Scholar]
- 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]
- Schober R., Nelson D. Fine structure and origin of amyloid deposits in pituitary adenoma. Arch Pathol. 1975 Aug;99(8):403–410. [PubMed] [Google Scholar]
- Shirahama T., Skinner M., Cohen A. S. Immunocytochemical identification of amyloid in formalin-fixed paraffin sections. Histochemistry. 1981;72(2):161–171. doi: 10.1007/BF00517130. [DOI] [PubMed] [Google Scholar]
- Tötterman K. J., Manninen V. Tumefactive liver infiltration in amyloidosis. Ann Clin Res. 1982 Feb;14(1):11–14. [PubMed] [Google Scholar]
- Wright J. R., Calkins E., Humphrey R. L. Potassium permanganate reaction in amyloidosis. A histologic method to assist in differentiating forms of this disease. Lab Invest. 1977 Mar;36(3):274–281. [PubMed] [Google Scholar]






