Recurrent Gastric Hemorrhaging with Large Submucosal Hematomas in a Patient with Primary AL Systemic Amyloidosis: Endoscopic and Histopathological Findings

Abstract

A 64-year-old woman who suffered intractable gastric ulcers with hemorrhaging showed huge submucosal hematomas in her stomach on the endoscopic examination. Since gastric mucosal biopsy revealed amyloid deposition and IgG type M protein was detectable in her serum, she was diagnosed as having primary AL systemic amyloidosis. The gastric hemorrhages did not improve despite intensive medication, so total gastrectomy was performed, resulting in an unfavorable outcome. Massive deposition of amyloid with A immunoreactivity was seen on the submucosal vessels in her stomach. This is a rare primary AL systemic amyloidosis case showing recurrent and fatal gastric submucosal hematomas.

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

“…This clinical picture resembles that of hemophilia. Recurrent submucosal hematomas in the gastrointestinal tract have also been reported in a patient with systemic AL amyloidosis (44). According to several recent reports, acquired hemostatic abnormalities, including coagulation factor deficiencies, hyperfibrinolysis and platelet dysfunction, can occasionally cause massive bleeding in patients with systemic AL amyloidosis (47,48); however, the pathological findings observed in the present study suggest that a hemorrhagic tendency is also ascribable to increased fragility and impaired vasoconstriction of the vessels affected by amyloid deposition.…”

“…Amyloid fibrils gradually accumulate in the GI wall and cause mucosal impairment leading to bleeding and intestinal pseudo-obstruction in parallel with a decrease in bowel motility via direct pressure of the neighboring tissues and/or ischemic changes due to vascular involvement (42,43). Endoscopically, mucosal polypoid lesions with friability and erosions are common findings (44,45). Due to the frequent involvement of the vascular walls, GI bleeding is more likely to occur in patients with systemic AL amyloidosis than in those with the localized form (31,45).…”

Increased plasmin‐α2‐antiplasmin levels indicate activation of the fibrinolytic system in systemic amyloidoses

“…Apparently, mechanic vessel rupture by localized accumulating amyloid causes bleeding. However, the bleeding tendency seen in most patients is not only related to vessel rupture by amyloid [14–16,19] and lacks a well‐defined cause.…”

Gastric Intramural Hematoma: A Case Report and Literature Review

“…A surgical approach has also been used in the management of gastric hematomas. However, in both of the reported cases, amyloidosis with vascular microaneurysms, and persistent and repetitive bleeding was identified (22,23). One of the two patients died after surgery as a result of multiorgan failure secondary to hypovolemic shock (22) while the second patient had a favourable postoperative course (23).…”