SummaryThe gastrointestinal tract is frequently in involved light-chain (AL) amyloidosis, but significant hemorrhagic complications are rare. A 71-year-old man presented to our hospital with dyspepsia and heartburn for 1 month. Gastroscopy revealed a large submucosal hematoma at the gastric fundus. Two days later, a follow-up gastroscopy indicated extensive expansion of the hematoma throughout the upper half of the stomach. The hematoma displayed ongoing expansion during the endoscopic examination, suggesting that rupture was imminent. Emergency total gastrectomy was performed, and amyloidosis was confirmed after examining the surgical specimen. Bone marrow examination revealed multiple myeloma, and serum immunoglobulin assay confirmed the diagnosis of myeloma-associated AL amyloidosis. At manuscript submission, the patient was doing well and was undergoing chemotherapy

Chae, Gi Bong

Hong, Seong Kweon

Kim, Song-Yi

Kim, Yang Hee

Lee, Seung Koo

Moon, Suk-Bae

Park, Sung-Bae

Elsevier - Publisher Connector 

Asian Journal of Surgery (2016) 39, 113e115Available online at www.sciencedirect.com
ScienceDirect
journal homepage: www.e-asianjournalsurgery.comCASE REPORTLight-chain amyloidosis presenting with
rapidly progressive submucosal hemorrhage
of the stomachSong-Yi Kim a,c, Suk-Bae Moon a,c, Seung Koo Lee b,
Seong Kweon Hong a, Yang Hee Kim a, Gi Bong Chae a,
Sung-Bae Park a,*aDepartment of Surgery, Kangwon National University Hospital, Kangwon National University School of
Medicine, Chuncheon, South Korea
bDepartment of Pathology, Kangwon National University Hospital, Kangwon National University School
of Medicine, Chuncheon, South KoreaReceived 19 August 2013; accepted 23 September 2013
Available online 15 November 2013KEYWORDS
amyloidosis;
hemorrhage;
stomach* Corresponding author. Departmen
Korea.
E-mail address: bsb1971_kr@kangw
c S.-Y.K. and S.-B.M. contributed eq
http://dx.doi.org/10.1016/j.asjsur.20
1015-9584/Copyrightª2013, Asian Sur
license (http://creativecommons.org/Summary The gastrointestinal tract is frequently in involved light-chain (AL) amyloidosis,
but significant hemorrhagic complications are rare. A 71-year-old man presented to our hospi-
tal with dyspepsia and heartburn for 1 month. Gastroscopy revealed a large submucosal hema-
toma at the gastric fundus. Two days later, a follow-up gastroscopy indicated extensive
expansion of the hematoma throughout the upper half of the stomach. The hematoma dis-
played ongoing expansion during the endoscopic examination, suggesting that rupture was
imminent. Emergency total gastrectomy was performed, and amyloidosis was confirmed after
examining the surgical specimen. Bone marrow examination revealed multiple myeloma, and
serum immunoglobulin assay confirmed the diagnosis of myeloma-associated AL amyloidosis.
At manuscript submission, the patient was doing well and was undergoing chemotherapy.
Copyright ª 2013, Asian Surgical Association. Published by Elsevier Taiwan LLC. This is an open
access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-
nd/4.0/).t of Surgery, Kangwon National U
on.ac.kr (S.-B. Park).
ually to this work.
13.09.013
gical Association. PublishedbyElse
licenses/by-nc-nd/4.0/).niversity Hospital, 156 Baekryoung-ro, Chuncheon 200-722, South
vier TaiwanLLC. This is anopenaccess article under theCCBY-NC-ND
114 S.-Y. Kim et al.1. Introduction
Amyloidosis describes a group of diseases that result from
the extracellular deposition of protein fibrils in tissues and
organs. Light-chain (AL) amyloidosis, also called primary
systemic amyloidosis, is usually caused by a clonal expan-
sion of plasma cells in the bone marrow. These cells secrete
a clonal immunoglobulin that deposits as amyloid fibrils in
tissues.1 Diagnosis of AL amyloidosis is straightforward
when a clonal plasma cell dyscrasia can be demonstrated.
However, diagnosis may be delayed until organ-specific
symptoms raise the suspicion of amyloidosis. The gastroin-
testinal tract is one of the most commonly affected organs,
but gastric involvement causing substantial bleeding has
rarely been reported in AL amyloidosis.2 In this study, we
describe a case of rapidly progressive gastric submucosal
hemorrhage and consequent total gastrectomy in a patient
with AL amyloidosis.Figure 1 (A) Initial gastroscopy indicated a very large sub-
mucosal hematoma from the gastric fundus to the anterior wall
of the high body. (B) Follow-up gastroscopy showed an exten-
sive submucosal hematoma covering the entire upper half of
the stomach.2. Case report
A 71-year-old man presented to our hospital with dyspepsia
and heartburn that had developed 1 month previously. His
family and medical histories were unremarkable. Physical
examination and laboratory tests revealed no significant
findings. Upon gastroscopy, a very large submucosal he-
matoma was noted from the fundus to the anterior wall of
the high body. The overlying mucosa was intact, and no
active bleeding was observed. Two days later, a follow-up
gastroscopy revealed an extensive submucosal hematoma
involving the upper half of the stomach (Fig. 1A and B).
Ongoing hematoma expansion was noted during the endo-
scopic examination, implying impending rupture. Biopsy
was not attempted to avoid hematoma rupture and cata-
strophic bleeding. Celiac angiography revealed diffuse
extravasation throughout the upper stomach and no foci for
embolization were identified. Although the patient was
hemodynamically stable at that time, surgery was recom-
mended to prevent further bleeding and sudden rupture of
the hematoma. Upon performing laparotomy, it was noted
that the submucosal hematoma had spread to the entire
gastric wall, and total gastrectomy was inevitable. Patho-
logical examination indicated the presence of amyloid fi-
brils and AL protein deposition in vessel walls, suggesting an
AL amyloidosis (Fig. 2A and B). The concentrations of serum
immunoglobulin kappa and lambda light chains were 7.91
and 552.65 mg/L, respectively, resulting in a kappa-to-
lambda ratio of 0.01. Bone marrow biopsy confirmed mul-
tiple myeloma, and the patient was diagnosed with primary
lambda-AL amyloidosis associated with multiple myeloma.
At his 8-month postoperative follow-up, the patient was
doing well and undergoing chemotherapy.3. Discussion
The gastrointestinal tract can be affected by any type of
amyloidosis, and biopsies identify gastric involvement in 8%
of amyloidosis cases. Only 1% of amyloidosis patients pre-
sent with gastrointestinal symptoms, which include nausea,
vomiting, epigastric pain, and gastric outlet obstruction.Hemorrhagic complications of amyloidosis with gastric
involvement are rare.3 Iijima-Dohi et al4 and Usui et al5
reported two cases of massive gastric bleeding in AL
amyloidosis; both patients were treated by emergency total
gastrectomy. As in the present study, those patients had no
predisposing factors for spontaneous bleeding, such as
coagulopathy, trauma, or peptic ulcer disease.
In gastrointestinal amyloidosis, the amyloids usually
deposit in the submucosa, and the mucosal architecture
remains normal until massive deposits of amyloid accumu-
late and destroy all mucosal structures.6,7 Yoshii et al8
suggested that colonic submucosal hematoma is a highly
suggestive finding of AL amyloidosis in the absence of other
endoscopic pathologies. Therefore, in patients with spon-
taneous gastric submucosal hemorrhage who are otherwise
healthy and without mucosal abnormalities, AL amyloidosis
with gastric involvement should be considered as a differ-
ential diagnosis.
Figure 2 Surgical specimen: (A) amorphous pinkish hyaline
deposits were observed in vessel walls (arrows; hematoxylin
and eosin stain, 400); (B) polarizing microscopy indicated a
greenish birefringence corresponding to hyaline deposits in the
same layer as (A) (arrowheads; Congo red stain, 400).
Amyloidosis and gastric submucosal hemorrhage 115Because the submucosal hematoma is susceptible to
touch bleeding, when AL amyloidosis is suspected, diag-
nostic tests should be individualized to avoid imprudent
endoscopic biopsy and significant bleeding. AL amyloidosis
is a systemic disease, and amyloid can be deposited in any
tissue of the body, although it most commonly deposits in
the fat of the abdominal wall.9 We performed total gas-
trectomy under emergent conditions without a clear diag-
nosis. However, considering the morbidity and invasiveness
of major gastrectomy, definite diagnosis should be made
preoperatively whenever possible.
The mainstay of AL amyloidosis treatment is the use of
drugs with a focus on targeting plasma cell diseases.However, confronted with hemorrhagic complications, the
first-line treatment should be selected cautiously. Although
the exact pathogenesis of submucosal bleeding in AL
amyloidosis remains unclear, the deposition of AL amyloid
fibrils within the intima or adventitia of the vessels con-
tributes to fragility and spontaneous bleeding beneath the
lamina propria.10 Because amyloid deposition within tissues
is irreversible and cannot be cleared by medical treatment
and because the natural course of AL amyloidosis is rapidly
progressive and fatal if untreated,1 we propose that hem-
orrhagic complications in AL amyloidosis should be
managed promptly by surgical intervention in cases with
massive hemorrhage.
In summary, we described a patient with AL amyloidosis
presenting with a rapidly progressive gastric submucosal
hemorrhage. An extensive submucosal hematoma necessi-
tated total gastrectomy, and the diagnosis was determined
postoperatively. It is important to differentiate AL
amyloidosis in the setting of spontaneous submucosal
hemorrhage without predisposing factors, and prompt
diagnostic and therapeutic interventions should be
initiated.
References
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Kasper DL, et al., eds. Harrison’s Principles of Internal Medi-
cine. 17th ed. New York, NY: McGraw-Hill; 2008:2145e2149.
2. Kim SH, Kang EJ, Park JW, et al. Gastrointestinal amyloidosis
presenting with multiple episodes of gastrointestinal bleeding.
Cardiovasc Intervent Radiol. 2009;32:577e580.
3. Baird-Howell MA, Wurzel J. Fatal gastrointestinal hemorrhage
in a paraplegic man with undiagnosed AA (secondary)
amyloidosis. Amyloid. 2011;18:245e248.
4. Iijima-Dohi N, Shinji A, Shimizu T, et al. Recurrent gastric
hemorrhaging with large submucosal hematomas in a patient
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pathological findings. Intern Med. 2004;43:468e472.
5. Usui M, Matsuda S, Suzuki H, Hirata K, Ogura Y, Shiraishi T.
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8. Yoshii S, Mabe K, Nosho K, et al. Submucosal hematoma is a
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two case reports. World J Gastrointest Endosc. 2012;4:
434e437.
9. Rajkumar SV, Gertz MA. Advances in the treatment of
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Light-chain amyloidosis presenting with rapidly progressive submucosal hemorrhage of the stomach 

The gastrointestinal tract is frequently in involved light-chain (AL) amyloidosis, but significant hemorrhagic complications are rare. A 71-year-old man presented to our hospital with dyspepsia and heartburn for 1 month. Gastroscopy revealed a large submucosal hematoma at the gastric fundus. Two days later, a follow-up gastroscopy indicated extensive expansion of the hematoma throughout the upper half of the stomach. The hematoma displayed ongoing expansion during the endoscopic examination, suggesting that rupture was imminent. Emergency total gastrectomy was performed, and amyloidosis was confirmed after examining the surgical specimen. Bone marrow examination revealed multiple myeloma, and serum immunoglobulin assay confirmed the diagnosis of myeloma-associated AL amyloidosis. At manuscript submission, the patient was doing well and was undergoing chemotherapy

Song-Yi Kim

Suk-Bae Moon

Seung Koo Lee

Seong Kweon Hong

Yang Hee Kim

Gi Bong Chae

Sung-Bae Park

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Asian Journal of Surgery

Light-chain amyloidosis presenting with rapidly progressive submucosal hemorrhage of the stomach

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Light-chain amyloidosis presenting with rapidly progressive submucosal hemorrhage of the stomach

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