1Department of Internal Medicine, PGIMER, Chandigarh, India.
2Department of Histopathology, PGIMER, Chandigarh, India.
The blood pressure was 130/80 mmHg and the pulse rate was 98 beats/minute. On physical examination, marked pallor, glossitis and angular cheilitis were found. The barium swallow test revealed esophageal stricture with an anterior shelf-like projection at the hypopharynx and cervical esophagus junction, suggestive of cricopharyngeal web (Fig. 1). The complete blood counts analysis showed hemoglobin level of 6.0 g/dL, white cells counts of 4.5×109/L, and platelet counts of 700×109/L. On peripheral blood smear, microcytic hypochromic red cells with aniso-poikilocytosis were remarkable with some pencil cells. The iron profile (serum iron, 31 mg/dL; iron-binding capacity, 435 mg/dL; transferrin saturation, 7%; and serum ferritin, 10 ng/mL) was consistent with iron deficiency anemia (IDA). The results of fecal occult blood, IgA-tissue transglutaminase antibody, anti-nuclear antibody, and urea breath test for
Amyloidosis is defined as deposition of amyloid (an amorphous homogenous fibrillar material produced as a consequence of protein misfolding and identified by its characteristic apple green birefringence on polarized light microscopy by using Congo red stain and beta-pleated appearance on electron microscopy) in the extracellular space, causing distortion of tissue architecture and function . Amyloidosis can be either systemic (the site of protein production is remote from its deposition) or localized (protein is produced at the site of deposition, with the usual sites being the respiratory tract, bladder, breast, skin, or gastrointestinal tract), the latter being a less common entity . Systemic amyloidosis can be classified as amyloid light-chain (AL) type, amyloid A (AA) type, or familial transthyretin-associated (ATTR) type . The symptoms are various depending on the organs involved. Because many gastrointestinal (GI) symptoms such as diarrhea, early satiety, and weight loss could be caused by other mechanisms unrelated to amyloidosis, the diagnosis of GI amyloidosis requires a biopsy-proven amyloid deposition in the GI tract .
GI amyloidosis is uncommon and is usually observed in systemic amyloidosis (7% of AL amyloidosis) . Of the 2,334 patients with amyloidosis evaluated by Cowan et al. , 76 (3.3%) had biopsy-proven GI amyloidosis, which was systemic in 60 patients (2.57%) and localized in 16 (0.68%). Inayat and Hurairah  reported a case of localized duodenal amyloidosis (secondary, AA deposition) presenting as unexplained IDA that improved with iron supplementation. Given the fact that rheumatoid arthritis is the most common cause of AA amyloidosis, the etiology of our case was chronic inflammation resulting from rheumatoid arthritis . As no evidence of GI bleeding was found on EGD, malabsorption was likely responsible for IDA.
In our opinion, presentation of localized GI amyloidosis (AA type) as Plummer-Vinson syndrome is exceptionally rare. This case highlights the importance of endoscopy in the initial workup of unexplained IDA and need for a biopsy even if no mucosal abnormality is identified. In addition, the differential diagnosis with systemic amyloidosis or plasma cell dyscrasia is also emphasized here, because localized amyloidosis usually could be controlled with conservative management.
No potential conflicts of interest relevant to this article were reported.
The barium swallow test in the anteroposterior