Gastrointesinal: Colonic amyloidosis causing submucosal haematoma and bleeding from strainingAmyloidosis is a rare disorder of extracellular deposition of an abnormal fibrillar protein, disrupting tissue structure and function. Primary amyloidosis (monoclonal immunoglobulin light chains, AL) is the most common form, which can be idiopathic or associated with plasma cell dyscrasias. Involvement in the gastrointestinal (GI) tract is very common among patients with primary amyloidosis, with the small and large intestines being the most frequently affected organs. Amyloid deposition is dominant in the muscularis mucosa, submucosa and muscularis propria, leading to polypoid protrusions, thickened folds or mucosal ulceration. As a result, GI manifestations of AL amyloidosis range widely from diarrhoea, constipation, intestinal obstruction (mechanical or ileus) to GI bleeding, including submucosal haematoma. The risk of GI bleeding in primary amyloidosis is further potentiated by the presence of a thrombin inhibitor and a deficiency of factor X. These coagulopathies can be found in up to 20% of patients with AL amyloidosis. As treatment of these coagulopathies with conventional fresh frozen plasma, platelets, or even plasma exchange is ineffective, great caution must be taken in the decision of mucosal biopsy in patients with AL amyloidosis to avoid a potentially refractory, difficult to treat GI bleeding.A 50-year old lady with a history of primary amyloidosis developed peri-rectal bleeding in the context of known gastro-intestinal involvement, proven on colonic biopsies obtained 1-year prior (Figure 1a-b, arrow indicates Congo stain positive). On this occasion, the patient described a 4-day history of severe constipation and the passage of frequent, large volume motions of bright red blood mixed with stool after significant straining. On examination, the patient had a resting pulse rate of 105 and blood pressure of 70/50 mmHg. Laboratory investigations revealed a haemoglobin drop from 102g/L to 87g/L over 3-days. Following resuscitation with packed red blood cells and fresh frozen plasma, a flexible sigmoidoscopy was performed that revealed a tubular, eccentric, semi-circumferential bluish submucosal abnormality that extended for 15cm in the proximal descending colon (Figure 2a-b). Mucosal pit pattern of colonic mucosa was noted on the slough overlying the bluish abnormality (Figure 2b). Oozing fresh blood was also noted from the edge of this abnormality, which is thought to be a colonic sub-mucosal haematoma from amylodosis ( Figure 2b). Biopsies were deemed unsafe as it can precipitate further bleeding. The bleeding stopped spontaneously after 3 days, with complete resolution of the haematoma on repeat flexible sigmoidoscopy performed 2 weeks later. Contributed by
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