THE AMERICAN JOURNAL OF GASTROENTEROLOGY
1, 2000
© 2000 by Am. Coll. of Gastroenterology
Published by Elsevier Science Inc.
Acute Small Bowel Pseudo-Obstruction Due to AL
Amyloidosis: A Case Report and Literature Review
Rachel N. Koppelman, B.S., Neil H. Stollman, M.D., F.A.C.P., F.A.C.G., Francisco Baigorri, M.D., and
Arvey I. Rogers, M.D., M.A.C.G.
Division of Gastroenterology, University of Miami School of Medicine, Miami, Florida
ABSTRACT
Amyloidosis may uncommonly present with intestinal pseu-
do-obstruction. Previous reports have described an acute
presentation with AA amyloid and a more chronic syndrome
with AL amyloid. We report the case of a 78-yr-old man
who presented with clinical and radiographic features of an
acute small bowel obstruction and who, at laparotomy, was
found to have intestinal pseudo-obstruction due to AL amy-
loidosis. We believe this case represents the first report of
acute pseudo-obstruction from AL amyloidosis; awareness
of this presentation may facilitate earlier diagnosis. (Am J
Gastroenterol 2000;95:294 –296. © 2000 by Am. Coll. of
Gastroenterology)
INTRODUCTION
Amyloidosis is a disorder marked by the extracellular dep-
osition in organs and tissues of amyloid, an insoluble fibril-
lar protein. Clinical manifestations of amyloidosis result
from involvement of many affected organ systems, espe-
cially renal, cardiac, neurological, or hematological. GI
manifestations are less common and, when they occur, may
include macroglossia, dysphagia, bleeding, or malabsorp-
tion. We report a patient who presented with an acute small
bowel obstruction and was found to have a pseudo-obstruction
due to deposition of AL amyloid protein in the small intestine.
CASE REPORT
The patient is a 78-yr-old African-American man who pre-
sented with decreased appetite, an unquantified weight loss,
dehydration, and lethargy over a number of months. The
patient denied abdominal pain, nausea, emesis, diarrhea, or
melena. He was admitted to the hospital for clinical dehy-
dration and anemia. Initial physical examination was unre-
markable. Given his anemia, and a history of prior bleeding
duodenal ulcer, a nasogastric lavage was performed, reveal-
ing a small amount of coffee ground material. An endoscopy
demonstrated severe erosive esophagitis and candidiasis.
The patient was hydrated and given cimetidine and flucon-
azole i.v. On hospital day 5, the patient developed signifi-
cant abdominal distention. An abdominal x-ray revealed
dilated loops of small bowel consistent with a small bowel
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PII S0002-9270(99)00771-6
obstruction (Fig. 1). The patient had no history of prior
surgery or any evidence of hernias.
The abdominal distention and x-ray abnormalities failed
to resolve after 24 h of conservative treatment with naso-
gastric suction and nothing by mouth, and a laparotomy was
performed on hospital day 6. The small bowel was noted to
be atonic and diffusely distended, with markedly thickened
walls which “felt like cardboard.” The distal ileum was
grossly normal, and there was no evidence of mechanical
obstruction. The bowel was viable by Doppler and fluores-
cein studies. A full thickness biopsy was obtained from the
abnormal small bowel, and the abdomen was closed. Congo
red stain revealed extensive amyloid deposition in the mus-
cularis propria and submucosal small vessels (Fig. 2A and
2B).
Further workup revealed an M-spike in the urine, which,
on immunofixation, was shown to be free monoclonal kappa
light chains. A bone marrow biopsy revealed ⬎ 90% plasma
cells, consistent with multiple myeloma.
DISCUSSION
Amyloidosis is a disorder marked by deposition of an ab-
normal proteinaceous substance (amyloid) between cells. It
may involve virtually any organ system in the body, leading
to severe pathophysiological consequences. Despite the
striking histological and morphological uniformity of the
amyloid protein in all cases, it is quite clear that amyloid is
not a single chemical entity. There are two major and
several minor biochemical forms, which are deposited by
different pathogenetic mechanisms. Amyloidosis, therefore,
should not be considered a single disease but, rather, a group
of diseases that share in common the deposition of similar-
appearing proteins. The deposited amyloid fibrils are ar-
ranged in a ␤-pleated sheet formation, which provides the
binding sites for the Congo red dye used for diagnosis. The
Congo red stain imparts a unique apple-green birefringence
when viewed with polarized light.
Amyloidosis can be separated into two major chemical
groups. Primary amyloidosis (AL) results from deposition
of immunoglobulin light chains or their fragments, produced
by aberrant clones of B cells. The best example is amyloid-
osis associated with multiple myeloma, a malignant plasma
AJG – January, 2000
Figure 1. Abdominal x-ray revealing dilated loops of small bowel
consistent with small bowel obstruction.
Figure 2. A: Hematoxylin and eosin stain (⫻4), revealing amyloid deposition in the muscularis propria and submucosal tissue. B: Congo
red stain (⫻10), revealing apple-green birefringence of a submucosal vessel.
Small Bowel Pseudo-Obstruction Due to AL Amyloidosis 295
cell neoplasm. In the United States this is the most common
form of amyloidosis. In contrast, the secondary amyloidoses
(AA) are caused by amyloid formed from serum amyloid A,
an acute phase protein produced in response to inflammation
(1). AA amyloidosis typically occurs in patients with rheu-
matoid arthritis, inflammatory bowel disease, and untreated
familial Mediterranean fever.
The presenting features of this disease are protean. Initial
symptoms are generally nonspecific, often including fatigue
and weight loss, but the diagnosis is rarely made at that
point. More specific symptoms may then ensue, reflecting
the main organ system involved. The organs most com-
monly affected are the kidneys and the heart (1). Renal
involvement may manifest as mild proteinuria or frank
nephrosis. The renal lesion is usually not reversible, and in
time leads to progressive azotemia and end-stage renal dis-
ease. Localized accumulation of amyloid may be noted in
the ureter, bladder, or other parts of the genitourinary tract
(2). Cardiac manifestations consist primarily of congestive
failure, cardiomegaly, and arrhythmias (2). Autonomic and
sensory neuropathies are also relatively common features,
often with a history of carpal tunnel syndrome and a sensory
296 Koppelman et al.
neuropathy that usually has a distal and symmetric pattern
(1).
The GI tract is less frequently affected, but may be
involved by both AA and AL amyloidoses. Symptoms may
result from direct involvement of the GI tract at any level or
from infiltration of the autonomic nervous system with
amyloid. Infiltration of the tongue results in macroglossia in
as many as 20% of cases of primary amyloidosis (3). When
not enlarged, the tongue may become stiffened and firm to
palpation. Esophageal involvement may decrease lower
esophageal sphincter pressure and alter esophageal peristal-
sis, resulting in reflux and/or dysphagia (4). Gastric infil-
tration may produce prominent gastric folds, gastric outlet
obstruction, ulcers, or bleeding (3). Amyloid deposition in
the small bowel may accumulate between the muscle layers
and compromise absorption or motility. Intestinal obstruc-
tion, perforation, infarction, nodules, ulcers, and bleeding
have all been described as a result of amyloid deposition (5).
Symptoms may include diarrhea, constipation, megacolon,
fecal incontinence, or rectal prolapse. Malabsorption may be
due to bacterial overgrowth, mucosal ischemia, exocrine
pancreatic insufficiency, or submucosal amyloid deposition,
which creates a physical barrier to absorption (6).
Intestinal dysmotility and pseudo-obstruction have been
described in a small number of amyloidosis patients. This
has been postulated to be caused by infiltration of either
smooth muscle (myopathy) or the myenteric plexus (neu-
ropathy). Tada et al. have suggested that AA and AL amy-
loid differ in their main intestinal deposition patterns and
consequent clinical presentations. Two AL patients with
chronic pseudo-obstruction syndromes have been described,
both of whom had extensive deposition of amyloid in the
muscularis propria (7). Acute pseudo-obstruction, on the
other hand, has been reported in 13 patients with AA amy-
loid, who were shown to have myenteric plexus deposition
without appreciable muscle infiltration (7). This suggests
that intestinal pseudo-obstruction in patients with amyloid-
osis is caused by either myopathy or neuropathy, depending
on the chemical type of amyloid. Our patient, with the AL
type of amyloidosis, had extensive infiltration of the mus-
cularis on biopsy, yet presented acutely. To our knowledge,
AJG – Vol. 95, No. 1, 2000
this is the first reported case of AL amyloid presenting with
an acute intestinal pseudo-obstruction.
In summary, intestinal amyloidosis may uncommonly
present with dysmotility and intestinal pseudo-obstruction
due to myopathy or neuropathy from protein deposition.
Previous reports have described a mainly myopathic process
in AL, with a chronic presentation, and a neuropathic (my-
enteric plexus) process in AA, with a more acute presenta-
tion. This distinction may be artificial and inaccurate, as our
patient, with AL and muscularis infiltration histologically
presenting with an acute pseudo-obstruction. Awareness of
these manifestations of amyloidosis may facilitate an earlier
diagnosis, and perhaps a more favorable prognosis, in these
patients.
Reprint requests and correspondence: Neil H. Stollman, M.D.,
1201 NW 16th Street, Gastroenterology, D-1007, Miami, FL
33125.
Received Sep. 3, 1998; accepted Dec. 11, 1998.
REFERENCES
1. Falk RH, Conenzo RL, Skinner M. The systemic amyloidoses.
N Engl J Med 1997;337:898 –909.
2. Cohen AS. Amyloidosis. In: Isselbacher RJ, Braunwald E,
Wilson JD, et al., eds. Harrison’s principles of internal med-
icine, 13th ed. New York: McGraw-Hill, 1994:1625–30.
3. Neil GA, Weinstock JV. GI manifestations of systemic dis-
eases. In: Yamada T, Alpers DH, Owyang C, et al., eds.
Textbook of gastroenterology, 2nd ed. Philadelphia: JB Lip-
pincott, 1995:2434 –5.
4. Rubinow A, Burakoff R, Cohen AS, et al. Esophageal ma-
nometry in systemic amyloidosis. Am J Med 1983;75:951–5.
5. Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and labo-
ratory features in 229 cases. Mayo Clin Proc 1983;58:665– 83.
6. Weber JR, Ryan JC. Effects on the gut of systemic disease and
other extraintestinal conditions. In: Feldman M, Scharschmidt
BF, Sleisinger MH, eds. Gastroenterology and liver disease,
6th ed. Philadelphia: WB Saunders, 1998:430.
7. Tada S, Iida M, Yao T, et al. Intestinal pseudoobstruction in
patients with amyloidosis: Clinicopathologic differences be-
tween chemical types of amyloid protein. Gut 1993;34:
1412–7.