Jejunal Amyloidosis


Step 1: View clinicals

A 70 year old lady presents with generalised weakness and fatigability for 8 months. She had no other complaints, while her medical and surgical histories are unremarkable. Her FBC is significant for a Hb of 5.4 g/dL, along with a low MCV, MCHC and MCH. Her electrolytes, liver functions, renal functions, and coagulation profiles are normal. Repeated questioning reveals a history of intermittent tarry colored stools. Initial and repeat upper GI endoscopies are normal, as is a colonoscopy.

Step 2: Order all relevant investigations

Barium Follow-Through

You realize that a barium series may obscure any overt bleeding, and potentially interfere with subsequent investigations.

Capsule Endoscopy

There is active bleeding from the proximal jejunum; the mucosa in that region appears nodular, polypoid, and eroded. The remainder of the jejunum shows thickening of the valvulae conniventes.

Push Enteroscopy & Biopsy

Congo red staining of jejunal biopsies reveals extensive, dense, homogenous deposits in the lamina propria and submucosa, with apple-green birefringence with polarized light; the hematoxylin stain reveals amorphous, salmon-pink, sparsely cellular hyaline material.

Serum Protein Electrophoresis

Serum protein electrophoresis and immunofixation reveal a monoclonal band of IgG lambda paraprotein.

Step 3: Select appropriate management

Blood Transfusion

Score: ★★☆