Gastrointest Radiol 16:133-136 (1991)


Radiology 9 Springer-VerlagNewYorkInc.1991

Amyloidosis of the Colon Presenting as Isehemie Colitis: A Case Report and Review of the Literature Thai D. Trinh, Bronwyn Jones, and Elliot K. Fishman The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA

Abstract. Review of the English literature revealed 21 cases describing abnormalities on barium enema secondary to amyloidosis of the colon. These cases were categorized as to frequency of specific barium enema findings and distribution within the colon. The most common radiologic findings were luminal narrowing (11 of 21), loss of haustrations (10 of 21), thickened mucosal folds (8 of 21), mucosal nodularity (8 of 21), and ulceration (6 of 21). The most frequent locations of disease within the colon were the descending and rectosigmoid portions (13 of 21). We present a case of primary amyloidosis that demonstrates the findings of bowel wall thickening and luminal narrowing on doublecontrast barium enema and computed tomography (CT). Pathologic examination in our case, in addition to similar observations from the literature, suggests that at least a part of the radiologic changes of colonic amyloid can be attributed to bowel ischemia. Key words: Colon, amyloidosis - Ischemic colitis, diagnosis.

The radiologic changes of amyloidosis involving the colon are rarely seen on imaging studies. In a review of the English literature, we found only 15 references (totaling 21 cases) describing colonic amyloid. Although the radiologic manifestations varied among these studies, the most common findings included loss of haustrations, bowel narrowing, mucosal thickening and nodularity, and occasional ulceration. The descending and rectosigmoid colon were most commonly affected. Address offprint requests to: Thai D. Trinh, M.D., Department

of Radiology, The Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21205, USA

However, nearly all of the previous reports described findings using single-contrast technique, and no reports, to our knowledge, described changes seen on computed tomography (CT). In this article, we report a patient with primary amyloidosis of the colon who presented with gastrointestinal bleeding. A double-contrast barium enema demonstrated luminal narrowing with nodular mucosal fold thickening, which correlated well with colonic wall thickening seen on CT.

Case Report A 67-year-old woman presented to the emergency room for evaluation of bilateral pedal edema of 2 weeks duration. She reported one episode of bright red blood per rectum 3 weeks prior to presentation and was found to have berne-positive stools and anemia. She declined admission at that time, but w a s admitted 3 days later following a second episode of hematochezia. The patient reported a 1-year history of anemia treated with iron supplements. Positive physical findings included macroglossia, dry bibasilar lung crackles, a grade II/VI systolic ejection murmur, and pitting edema to mid-calf. Admission laboratory data were normal except for a hemoglobin level of 7.6 g/100 ml and a hematocrit of 23% with hypochromic and normocytic indices. Serum iron, transferrin, vitamin B12, haptoglobin, hemopexin, and coagulation parameters were normal. Urine analysis was negative for protein by dipstick, but 3 + protein was demonstrated by 5% sulfosalicylic acid precipitation. Air contrast barium enema revealed luminal narrowing with nodular, thickened mucosa involving a 25-30-cm long segment from the mid-descending to mid-sigmoid colon. The differential diagnosis included ischemic colitis or lymphomatous infiltration (Fig. 1). CT with oral and intravenous contrast revealed marked bowel wall thickening involving the left colon from the splenic flexure to the rectosigmoid, with luminal narrowing in the sigmoid colon (Fig. 2A and B). Flexible sigmoidoscopy revealed three sessile lesions at 27 cm and a nearby small area of hemorrhagic ulceration and mild mucosal edema with friability. Biopsies revealed vascular amyloidosis with adjacent ulceration and fresh hemorrhage (Fig. 3).


T.D. Trinh et al, : Amyloidosis of the Colon

Fig. 1. Air-contrast barium enema shows a 25-30-cm long luminal narrowing with submucosal nodularity and thickening involvingthe descending and sigmoid colon. Fig. 2. A, B CT of the pelvis after oral and intravenous contrast material demonstrates bowel wall thickening in the descending colon (white arrow) and bowel wall thickening with luminal narrowing in the sigmoid colon (black arrows). Fig. 3. Microscopic section from the mid-proximal sigmoid colon (congo red stain; original magnification, x 40) reveals amorphous deposits of amyloid in the submucosa and in the blood vessel walls, which show characteristic green birefringence under polarized light (arrows).

T.D. Trinh et al. : Amyloidosis of the Colon Urine protein electrophoresis showed spillage of monoclonal lambda light chain and monoclonal IgG without corresponding light chain. Serum protein electrophoresis was not obtained. Bone marrow biopsy was negative and bone survey was negative for lytic lesions. The patient was treated with blood transfusion and medical management. Her condition improved and she was discharged with the diagnosis of primary amyloidosis with resulting gastrointestinal hemorrhage.


Amyloidosis is a relatively uncommon disease characterized by the extracellular deposition of an insoluble fibrillar protein in various tissues of the body. The etiology is as yet unknown, although there appears to be a clear relationship between amyloidosis and abnormalities in the immune system [1-3]. Many classifications of amyloidosis have been proposed [4]. In general, however, amyloidosis can be divided into two categories - systemic and localized. The systemic category includes primary amyloidosis (the idiopathic form) and secondary amyloidosis (the reactive form that occurs in association with a number of chronic inflammatory or necrotizing diseases, such as tuberculosis, osteomyelitis, ulcerative colitis, rheumatoid arthritis, or certain neoplasms). This category also includes amyloidosis associated with multiple myeloma or other plasma cell dyscrasias, as well as the familial types of amyloidosis. The localized type of amyloidosis is site-limited and does not progress to systemic involvement. Our patient is felt to have primary amyloidoSis. Infiltration of the gastrointestinal tract with amyloid, as demonstrated by microscopic examination, is not unusual. Symmers [5] reported gastrointestinal tract involvement in 70% of autopsy cases of primary amyloidosis, whereas Dahlin [6] reported involvement in 55% of autopsy cases of secondary amyloidosis. Seventy-five percent of rectal biopsies were positive for amyloid in a study of 200 patients by Blum and Sohar [7]. Histopathologically, deposits of amyloid in the gastrointestinal tract are found in two locations: 1) in and around the walls of the small submucosal blood vessels and 2) within the mucosal layer and/ or within the muscular layers of the gut wall [8]. Deposition around the visceral nerves is also seen. In most cases, the submucosal blood vessels are the earliest and most frequent sites of amyloid deposition. Gilat et al. found that in primary amyloidosis, deposition was predominantly within the muscular layers, whereas in secondary amyloidosis, deposition was predominantly within the mucosal layer [9]. Involvement of the submucosal ves-

135 Table 1. Colonic abnormalities in 21 patients with amyloidosis* Barium enema findings


Luminal narrowing Loss of colonic haustrations Thickened mucosal folds Nodular mucosa (thumbprint, cobblestone) Ulceration Irregular or exaggerated haustra Dilatation Abnormal motility Mass lesion Bowel wall thickening

11 10 8 8 6 4 4 2 2 1

Areas of involvement were the ascending colon (3), transverse colon (3), descending colon (7), rectosigmoid (13), and diffuse or multifocal (3). Ref. [19] included two cases, but only one had sufficient detail to be used in this table. * See refs. [8, 12-25].

sels with amyloid can lead to blood vessel narrowing or occlusion with resulting ischemia or infarct. Cases of bowel perforation secondary to amyloidinduced ischemia have been reported [10, 11]. The biopsy material from our case demonstrated vascular amytoid deposition. It was felt, based on the histopathology from our case, that the radiographic changes seen on both double-contrast barium enema and CT were the result of bowel ischemia. Although the histologic details were not completely provided in all the references, four literature cases showed predominately vascular amyloid deposition [12-15]. In these instances, the radiographic changes seen can, as in our case, probably be ascribed to bowel ischemia. In addition, detailed radiologic-pathologic examination by Seliger et al. found similar correlation between changes on single-contrast barium enemas and the amount of muscular and, particularly, vascular amyloid deposition [8]. Amyloid deposition in the colon, although common on biopsy specimens, is rarely radiographically evident, and most patients with positive rectal biopsies have normal barium enema studies. When radiologic changes in the colon are present, however, they are varied. In our review of the literature, we found only 21 cases of amyloidosis involving the colon resulting in abnormal barium enema studies. Using the literature cases, we categorized the barium enema findings, according to frequency of observation, as well as distribution within the colon (Table 1). With the exception of one case, all were single-contrast studies. The most common abnormal findings included luminal narrowing, loss of colonic haustrations, thickened mucosal folds,


nodular mucosal pattern, and ulceration. One study reported progression of disease on three barium enema examinations over a 4-year period. The first study showed diminished haustration and thickened mucosal folds; the second study showed loss of haustration and atony; and the third study, luminal narrowing with nodular mucosa [12]. Our case also revealed colonic narrowing and thickened nodular mucosa. However, in our case, examination was with an air-contrast barium enema and the mucosal abnormalities were easily appreciated due to this technique's enhanced visualization of mucosal detail. Although no ulcerations were seen by barium enema, small ulcerations were present on sigmoidoscopy and on biopsy specimeff. Our patient also underwent CT examination of the abdomen and this demonstrated marked bowel wall thickening of the left colon with distal luminal narrowing and correlated well with the enema study. To our knowledge, this is the first reported example of colonic amyloidosis seen on CT. Interestingly, bowel wall thickening, which was the prominent finding seen on CT, is an unusual barium enema finding in the literature. This undoubtedly reflects the fact that the barium enema study only indirectly provides information about the bowel wall. In terms of location, the descending colon and rectosigmoid colon were the most common areas to demonstrate radiographic abnormalities. Diffuse colonic involvement and " s k i p " involvement were less frequently seen. Our case, which revealed changes in the descending and rectosigmoid colon on both barium enema and CT, correlated well with the literature. The radiographic manifestations of colonic amyloid, described in the literature and corroborated by our case, are nonspecific and appear to result in part from ischemia and not from amyloid deposition directly. Differentiation from other causes of ischemic colitis (thrombosis, embolism, low flow, and irradiation), as well as inflammatory bowel disease (particularly chronic ulcerative colitis) must be made.

References 1. Scott PP, Scott WW, Siegelman SS. Amyloidosis: a review. Semin Roentgenol 1986; 21:103-112 2. Kyle RA, Bayrd ED. Amyloidosis: a review of 236 cases. Medicine 1975 ; 54: 271-299

T.D. Trinh et al. : Amyloidosis of the Colon 3. Glenner GG, Ein D, Terry WD. The immunoglobulinorigin of amyloid. Am J Med 1972; 52:141-147 4. Benditt EP, Cohen AS, Costa PP, et al. Guidelines for nomenclature. In: Glenner GG, Costa PP, Freitas AF, eds. Amyloid amyloidosis. Amsterdam: Exerpta Medica, 1980:11-12 5. Symmers WSC. Primary amyloidosis: a review. J Clin Pathol 1956; 9:187-211 6. Dahlin DC. Secondary amyloidosis. Ann Intern Med 1949; 31:105-119 7. Blum A, Sohar E. The diagnosis of amyloidosis-ancillary procedures. Lancet 1962; 1:721 724 8. Seliger G, Krassner RL, Beranbaum ER, Miller F. The spectrum of roentgen appearance in amyloidosis of the small and large bowel: radiologic-pathologic correlation. Radiology 1971 ; 100:63-70 9. Gilat T, Revach M, Sohar E. Deposition of amyloid in the gastrointestinal tract. Gut 1969; 10: 98-104 10. Gilat T, Spiro HM. Amyloidosis of the gut. Am J Dig Dis 1968; 13:619-633 11. Akbarian M, Fenton J. Perforation of small bowel in amyloidosis. Arch Intern Med 1964; 114: 815-821 12. Ritvo M, Litner C. Roentgen manifestations of primary amyloidosis of the colon. A JR 1963; 89:760-765 13. Casad DE, Bocian JJ. Primary systemic amyloidosis simulating acute idiopathic ulcerative colitis. Am J Dig Dis 1965 ; 10 : 63-74 14. Wang CC, Robbins LL. Amyloid disease - its roentgen manifestations. Radiology 1956; 66: 489-501 15. Mallory A, Struthers JE, Kern F. Persistent hypotension and intestinal infarction in a patient with primary amyloidosis. Gastroenterology 1975; 68 : 1587-1592 16. Perarnau JM, Raabe JJ, Courrier A, et al. A rare etiology of ischemic colitis-amyloid colitis. Endoscopy 1982; 14:10%109 17. Legge DA, Carlson HC, Wollaeger EE. Roentgenologic appearance of systemic alnyloidosis involving gastrointestinal tract. A JR 1970; 110:406-412 18. Brom B, Bank S, Marks IN, et al. Ischemic colitis, gastric ulceration, and malabsorption in a case of primary amyloidosis. Gastroenterology 1969; 57:319-323 19. Yoshida T, Kanbe H, Haraguchi Y, et al. Significance of abnormal gastrointestinal gas in systemic amyloidosis. Am J Gastroenterol 1982; 77:917-921 20. Carlson HC, Breen JF. Amyloidosis and plasma cell dyscrasias: gastrointestinal involvement. Semin Roentgenol 1986; 21 : 128-138 21. Hodgson JR. Miscellaneous diseases of the colon. Semin Roentgenol 1968; 3 : 94-112 22. Johnson DH, Guthrie TH, Tedesco F J, et al. Amyloidosis masquerading as inflammatory bowel disease with a mass lesion simulating a malignancy. Am J Gastroenterol 1982; 77 : 141-145 23. Chernenkoff RM, Costopoulos LB, Bain GO. Gastrointestinal manifestations of primary amyloidosis. CMAJ 1972; 106:567-569 24. Bergman F. Amyloid "tumour" in sigmoid colon. Acta Pathol Microbiol Scand 1962; 55 : 395-400 25. Pear BL. Radiographic studies of amyloidosis. CRC Crit Rev Radiol Sci 1972; 3:425-452 Received: May 3, 1990; accepted." June 4, 1990

Amyloidosis of the colon presenting as ischemic colitis: a case report and review of the literature.

Review of the English literature revealed 21 cases describing abnormalities on barium enema secondary to amyloidosis of the colon. These cases were ca...
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