Recurrence of paraproteinemic crystalline keratopathy after corneal transplantation: A case of monoclonal gammopathy of ocular significance

American Journal of Ophthalmology Case Reports

Benno Kusters¹  Johannes R.M. Cruysberg²  Siamak Nobacht³  Gerard A. Rongen³  Myrte B. Breukink⁴ 

[1] Corresponding author. Department of Ophthalmology, Radboud University Medical Center, PO Box 9101, 6500, HB, Nijmegen, the Netherlands.;Department of Internal Medicine, Radboud University Medical Center, Nijmegen, the Netherlands;Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands;Department of Pathology, Radboud University Medical Center, Nijmegen, the Netherlands;

关键词: Crystalline keratopathy;    Paraproteinemia;    Monoclonal gammopathy;    Undetermined significance;    Ocular significance;   

DOI  :  

来源: DOAJ

【 摘 要 】

Purpose: To report the long-term follow-up (12 years) of a 36-year-old male patient with crystalline keratopathy of both eyes, diagnosed with monoclonal gammopathy of undetermined significance (MGUS). Complete ophthalmic, systemic, and corneal immunohistochemical evaluations were performed. Observations: Slit-lamp examination revealed bilateral fine iridescent confluent crystalline deposits in all layers of the cornea, both peripherally and centrally. Systemic evaluation revealed abnormal M protein, IgG-kappa type, in blood and urine. Bone marrow aspiration showed a monoclonal plasma cell concentration of 2%. Consequently, the patient was diagnosed with MGUS. Because of progressive bilateral visual loss in the following 10 years, a perforating keratoplasty was performed on the left eye. Immunohistochemical analysis of the native cornea (the corneal button) revealed depositions of the same M protein type as detected in plasma and urine. Electron microscopy showed rhomboid-shaped corneal deposits of various sizes up to 4 μm. Recurrence of crystalline keratopathy was observed 9 months after keratoplasty. The monoclonal protein remained stable and the MGUS did not progress to multiple myeloma nor a related disorder. Conclusions and importance: Crystalline keratopathy may be associated with MGUS in otherwise healthy individuals. If the keratopathy causes binocular visual loss, a corneal transplantation may be required. Unfortunately, recurrence of crystalline deposits in the corneal graft may occur within one year. This suggests that patients with vision impairment due to paraproteinemic keratopathy who are diagnosed as MGUS, in fact, have a monoclonal gammopathy of ocular significance (MGOS).

【 授权许可】

Unknown