Paraproteinemic Crystalline Keratopathy

Ophthalmic manifestations of atypical IgD multiple myeloma

“…Generalised corneal haze,9 prominent corneal nerves and reduced corneal sensitivity,10 vortex keratopathy,11 intracorneal copper deposits12 13 and crystals dispersed centrally and peripherally throughout all levels of the cornea14 15 have all previously been reported. Such corneal crystals have been shown to be deposits of monoclonal IgG kappa9 11 13–15 or lambda14 immunoglobulin, and may cause such visual morbidity to require penetrating keratoplasty 16. Confocal microscopy studies have shown these infiltrates to be hyperreflective globules17 or needle-like structures18 which are typically located in the corneal epithelium and anterior stroma, obscuring the normal corneal architecture but being reduced in size and number following chemotherapeutic treatment 17 18.…”

“…Confocal microscopy studies have shown these infiltrates to be hyperreflective globules17 or needle-like structures18 which are typically located in the corneal epithelium and anterior stroma, obscuring the normal corneal architecture but being reduced in size and number following chemotherapeutic treatment 17 18. Unfortunately, it has been reported that these crystals may recur in a corneal graft 14 16…”

Corneal Structural Changes in Nonneoplastic and Neoplastic Monoclonal Gammopathies

“…Different mechanisms have been proposed regarding the ways through which, in myeloma patients, serum proteins reach the cornea. In particular, transport via tear film, 13 diffusion from the aqueous fluid, 15 and increased permeability of the paralimbal vascular arcades 42 have been suggested. As to the epithelium, the cells showed, in both groups of gammopathic patients, a significantly increased optical density, similar to what has been demonstrated in dry eye.…”

“…Corneal transplantation, generally with a good outcome, 14 can be required in the course of gammopathies when severe corneal opacity occurs; however, a recurrence can develop in patients in whom the systemic disease is not controlled by therapy. 15,16 The corneal lesions occurring in monoclonal gammopathies have been described using in vivo confocal microscopy (IVCM) only in single case reports, 7,13,[17][18][19][20] examining patients with clinically evident corneal opacities determining alterations of visual acuity. The main confocal features were demonstrated throughout the corneal thickness, showing needle-shaped, hexagonal, or round deposits with crystalline appearance, 7,17,20,21 corresponding on the histopathologic exam to intra-and extracellular tubules, fibrils, and crystals.…”

“…The main confocal features were demonstrated throughout the corneal thickness, showing needle-shaped, hexagonal, or round deposits with crystalline appearance, 7,17,20,21 corresponding on the histopathologic exam to intra-and extracellular tubules, fibrils, and crystals. 9,14,15,22 The lesions occurred either before or after the systemic manifestations of the disease.…”