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 59-year-old Chinese female was referred to the chest clinic of the First Affiliated Hospital, College of Medicine, Zhejiang University (Hangzhou, China) in April 2011 due to a two-month history of cough, hemoptysis and general fatigue. A CT scan of the chest revealed a nodule in the left lower lung with mild enhancement in the lesion. The nodule progressed during the two-month follow-up period under treatment with anti-infection drugs. No other significant history was noted. The patient’s physical examination was unremarkable, and hematological and biochemical parameters were within normal limits.\nA dual phase FDG PET/CT scan was performed following six hours of fasting. FDG (5.5 MBq/kg) was injected intravenously through an antecubital vein while the patient remained at rest. Image acquisition was subsequently conducted using a Siemens Biograph 16 PET-CT scanner (Siemens Medical Solutions USA, Inc., Malvern, PA, USA). The PET/CT images revealed a 1.83×1.40 cm slightly lobulated nodule, with burr-like margins in the left lower lung, exhibiting moderately increased F-18 FDG uptake (maximum SUV of 2.6) in the initial images (1 h following the FDG injection), and more intense FDG uptake with an SUV of 3.5 (26.9% increase) in the delayed images (2 h following the injection) (). Based on the dual phase FDG PET/CT imaging findings, morphological features, contrast-enhanced chest CT imaging and medical history (progression of the nodule during the two-month follow-up period), lung malignancy was highly suspected. A percutaneous CT-guided thoracoscopic biopsy was subsequently performed. Histological examination revealed that the specimens contained amorphous, homogeneous material with a number of polyclonal plasma cells, lymphocytes and giant cells. Eosinophilic material exhibited apple-green birefringence under polarizing microscopy. Immunohistochemically, congo red staining was positive, and trichrome staining was negative, confirming the deposition of amyloid within the specimen (). Therefore, a diagnosis of primary nodular parenchymal pulmonary amyloidosis was determined, and the patient was discharged without chemotherapy and other treatment. After May 2011, the patient was followed up every 3 months and was in good clinical condition at the time of writing.