The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut

Simms, Larnelle N.; Suarez, Laura; Deeb, Khaled; Proenza, Jose

doi:10.1177/2050313x211040018

Cited by 2 publications

(1 citation statement)

References 15 publications

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“…A potential explanation is that AL amyloidosis is associated with coagulation disorders (5). Another potential reason is that amyloid deposits intruding on the perivascular and vascular walls make these walls fragile, impairing vasoconstriction and causing mucosal erosion and bleeding (6). Intestinal hemorrhage may stop spontaneously without intervention but tends to relapse, requiring endoscopic hemostasis or surgery (2).…”

Section: Discussionmentioning

confidence: 99%

Systemic immunoglobulin light chain (AL) amyloidosis initially presenting as hematochezia: a case report

Wang¹,

Yue²,

Chen³

et al. 2023

Ann Palliat Med

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Background: Amyloidosis, an systemic disorder featuring an accumulation of misfolded proteins, is a significant diagnostic challenge because of its broad range of symptoms. The symptoms of amyloidosis vary depending on the affected organs. Amyloid accumulation in the kidney generally manifests as proteinuria or impaired kidney function, whereas cases with gastrointestinal (GI) involvement present as abdominal pain, weight loss, or GI bleeding.Case Description: We report a case of systemic immunoglobulin light chain (AL) amyloidosis involving the colon and kidney in a 75-year-old female who presented with intermittent lower abdominal pain and hematochezia. A colonoscopy revealed multiple ulcerations and a submucosal hematoma with κ light chain deposition confirmed by biopsy. The patient had many comorbidities, including renal tuberculosis, chronic kidney disease, diabetes, coronary heart disease (CHD), and paroxysmal atrial fibrillation, which rendered her clinical manifestations confusing. Her condition was relatively stable during treatment with bortezomib and dexamethasone for 4 cycles.Conclusions: Systemic amyloidosis usually has a poor prognosis since most cases are detected in the late disease phase. Early disease detection depends on a comprehensive understanding of the disease and a keen recognition of the lesion. We suggest that in patients with hematochezia, colonic ulcer, and submucosa hematoma, amyloidosis with colonic involvement should be considered when other diseases are excluded.

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Section: Discussionmentioning

confidence: 99%

Systemic immunoglobulin light chain (AL) amyloidosis initially presenting as hematochezia: a case report

Wang¹,

Yue²,

Chen³

et al. 2023

Ann Palliat Med

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Surgical strategies for localized colorectal amyloidosis

Tahara¹,

Kohyama²,

Nakamitsu³

et al. 2023

surg case rep

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Background Localized colorectal amyloidosis has a good prognosis, but cases involving bleeding or perforation may require surgery. However, there are few case reports discussing the differences in the surgical strategy between the segmental and pan-colon types. Case presentation A 69-year-old woman with a history of abdominal pain and melena was diagnosed with amyloidosis localized in the sigmoid colon by colonoscopy. Since preoperative imaging and intraoperative findings could not rule out malignancy, we performed laparoscopic sigmoid colectomy with lymph-node dissection. Histopathological examination and immunohistochemical staining revealed a diagnosis of AL amyloidosis (λ type). We diagnosed localized segmental gastrointestinal amyloidosis, because there was no amyloid protein in the margins, and the tumor was localized. There were no malignant findings. Conclusions Unlike systemic amyloidosis, localized amyloidosis has a favorable prognosis. Localized colorectal amyloidosis can be classified into the segmental type, in which amyloid protein is deposited locally, and the pan-colon type, in which amyloid protein is deposited extensively in the colon. Amyloid protein causes ischemia due to vascular deposition, weakening of the intestinal wall due to muscle layer deposition, and decreased peristalsis due to nerve plexus deposition. No amyloid protein should remain outside the resection area. The pan-colon type is often reported to cause complications such as anastomotic leakage, and primary anastomosis should be avoided. On the other hand, if there is no contamination or tumor remnants in the margin, the segmental type may be considered for primary anastomosis.

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The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut

Cited by 2 publications

References 15 publications

Systemic immunoglobulin light chain (AL) amyloidosis initially presenting as hematochezia: a case report

Systemic immunoglobulin light chain (AL) amyloidosis initially presenting as hematochezia: a case report

Surgical strategies for localized colorectal amyloidosis

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