A 19-year-old healthy Indian female noted blurred vision, redness, and pain in the right eye (RE) for 6 months. On examination, visual acuity (VA) was counting fingers in RE and 20/20 in left eye (LE). The RE demonstrated shallow anterior chamber with 360 degrees peripheral anterior synechiae. Approximately 400 translucent spherules of 50-200 micron diameter were embedded in the iris stroma and anterior chamber angle [Fig. and ]. Other findings included posterior subcapsular cataract, elevated intraocular pressure (IOP) of 30 mm Hg, and advanced glaucomatous damage with C:D ratio of 0.9:1. The LE was normal. Iris biopsy demonstrated acellular, amorphous hyaline deposits within the iris stroma, weakly staining with Congo red, suggestive of amyloid []. Transmission electron microscopy (TEM) confirmed amyloid fibrils []. Results of analysis with in situ hybridization for subtyping were inconclusive due to the presence of heavily melanized melanocytes. Systemic evaluation including complete blood count, liver and kidney function tests, serum and urine protein electrophoresis, serum and urine light chain protein, bone marrow biopsy, and PET CT scan were normal. Over the following 3 years, the patient developed cataract, uncontrolled glaucoma, and corneal decompensation, requiring cataract surgery, glaucoma valve surgery, and penetrating keratoplasty, with final VA at 20/30 in affected eye [Fig. and ].
A 67-year-old male ex-smoker (>20 years) with a 30 pack-year history was investigated for morning cough and clear sputum with no constitutional symptoms and an active lifestyle. Past medical history included chronic obstructive pulmonary disease and ischaemic heart disease. External physical examination was unremarkable. Chest computed tomography (CT) showed a right middle lobe nodule which increased from 11 mm to 16 mm over six months. A positron emission tomography (PET) scan showed low radiolabeled [18F]-2-fluoro-2-deoxy-D-glucose (FDG) uptake with a standardised uptake value (SUV) max of 2.0 and no evidence of metastatic disease (). CT-guided biopsy identified eosinophilic amorphic material, which was Congo red negative, with no definitive diagnosis reached. Due to the volume increase of the suspicious lesion, a right middle lobe lobectomy was performed.