[Paraproteinemic Keratopathy as a Clinical Sign of Monoclonal Gammopathy]. / Paraproteinämische Keratopathie als klinisches Zeichen einer monoklonalen Gammopathie.

Patients with monoclonal gammopathy can show paraproteinemic keratopathy (PPK) with an indication to treatment. PPK has to be differentiated from corneal dystrophies, systemic metabolic disorders with corneal involvement, as well as from immunologic and inflammatory corneal diseases.

Recurrence of paraproteinemic keratopathy after penetrating keratoplasty and its assessment with confocal microscopy.

PURPOSE: To report on a case of recurrence of paraproteinemic keratopathy (PPK) associated with monoclonal gammopathy after bilateral penetrating keratoplasty. OBSERVATIONS: Penetrating keratoplasty was performed on both eyes of a 45-year-old man due to bilateral progressive corneal stromal clouding. Recurrence of the corneal stromal opacities accompanied by a decrease in visual acuity was observed on slit-lamp examination already two years after penetrating keratoplasty. Confocal laser scanning microscopy (CLSM) of the corneal grafts performed three years after penetrating keratoplasty showed bilateral morphological changes identical to that found in the patient's corneas prior to penetrating keratoplasty. A hematological work-up revealed monoclonal gammopathy of type IgG kappa. The histochemical examination of the explanted corneas confirmed the diagnosis of PPK. CONCLUSIONS AND IMPORTANCE: Paraproteinemic keratopathy is an underdiagnosed ophthalmological condition, which may be associated with potentially life-threatening hematologic disorders. A hematological workup should be performed in patients with corneal opacities of uncertain etiology. Penetrating keratoplasty should be performed with caution in patients with monoclonal gammopathy due to the possibility of a very fast recurrence of PPK in the corneal graft. This is the first presentation of the recurrence of flake-like PPK after penetrating keratoplasty assessed with CLSM.

[Hematological diagnosis in the corneal consultation]. / Hämatologische Diagnose in der Hornhautsprechstunde.

This case report describes a diagnostic chain in a patient with atypical bilateral corneal opacity, which led to the diagnosis of a hematological disorder. The patient's medical history, clinical appearance and findings in confocal microscopy gave rise to the suspicion of a paraproteinemic keratopathy. The hematological laboratory diagnostics revealed a monoclonal gammopathy of the IgG kappa type. The bone marrow puncture led to the diagnosis of a lymphoplasmacytic lymphoma, which belongs to the group of B­cell non-Hodgkin's lymphomas. This case demonstrates that paraproteinemic keratopathy can be associated with potentially life-threatening hematological diseases.

Peripapillary Choroidal Thickness and Choroidal Area in Glaucoma, Ocular Hypertension and Healthy Subjects by SD-OCT.

BACKGROUND: The exact pathogenesis of open angle glaucoma and ocular hypertension remains unclear. Hemodynamic influences are discussed as potential risk factors and the choroid may play an important role in the pathogenesis of open angle glaucoma or ocular hypertension. The current study investigates peripapillary choroidal thickness and choroidal area in patients with open angle glaucoma, subjects with ocular hypertension and healthy subjects using spectral-domain OCT. It furthermore assesses the association between peripapillary choroidal thickness and age, central corneal thickness, refractive error and intraocular pressure. PATIENTS AND METHODS: Prospectively recorded data of 213 eyes of 177 open angle glaucoma patients, 73 eyes of 50 subjects with ocular hypertension and 152 eyes of 116 healthy control subjects were analyzed by fitting a linear mixed model including age and disease. RESULTS: Peripapillary choroidal thickness was thinnest in glaucoma patients (125 µm), followed by healthy subjects (127 µm) and ocular hypertensive subjects (135 µm). A marginally significant difference was present between ocular hypertension and glaucoma (p=0.059). Thickest choroids were found superiorly and thinnest choroids inferiorly. Choroidal area was highest in the supero-nasal and lowest in the infero-temporal sectors. Choroidal thickness decreased with age, no significant correlation was evident between peripapillary choroidal thickness and refractive error or intraocular pressure. Peripapillary choroidal thickness and central corneal thickness are significantly negative correlated in healthy subjects. CONCLUSIONS: There is a trend towards thicker choroids in ocular hypertensive subjects compared to healthy subjects or glaucoma patients. Thickest choroids are found superiorly, thinnest inferiorly. Interestingly, choroidal area is thinnest in the temporal-inferior sector, one of the regions where glaucomatous damage tends to start.