Characterization of corneal deposition keratopathy in the setting of blood cell dyscrasia and a minimally invasive technique to clear the cornea in a single case

Stiefel, Hillary C.; Sandhu, Rasanamar K.; Miller, Audra K.; Wilson, David J.; Chamberlain, Winston

doi:10.1016/j.ajoc.2018.12.010

Cited by 8 publications

(10 citation statements)

References 15 publications

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“…In the literature, AS-OCT of paraproteinemic keratopathy has been described in 11 patients [ 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 ], with hematological diagnoses of MGUS [ 6 , 7 , 8 , 9 , 10 ], biclonal gammopathy of undetermined significance [ 7 ], smoldering multiple myeloma [ 11 ], multiple myeloma [ 9 , 12 ], and CLL [ 13 ]. The features of AS-OCT in reported cases of paraproteinemic keratopathy are summarized in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

Diagnosing Paraproteinemic Keratopathy: A Case Report

Mok

Kam

Aldave

et al. 2021

Case Rep Ophthalmol

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A 65-year-old man presented with bilateral, painless, progressive blurring of vision over 9 years. Slit-lamp examination revealed bilateral subepithelial corneal opacities in clusters located at the mid-periphery. Anterior segment optical coherence tomography, in vivo confocal microscopy (IVCM), serum protein electrophoresis, and molecular genetic testing were performed to evaluate the cause of corneal opacities. Anterior segment optical coherence tomography revealed a band-like, hyperreflective lesion in the Bowman layer and anterior stroma of both corneas. IVCM revealed hyperreflective deposits in the epithelium, anterior stroma, and endothelium. Serum protein electrophoresis identified the presence of paraproteins (immunoglobulin kappa), and molecular genetic testing revealed absence of mutations in the transforming growth factor beta-induced gene (<i>TGFBI</i>) and collagen type XVII alpha 1 gene (<i>COL17A1</i>). The ocular diagnosis of paraproteinemic keratopathy eventually led to a systemic diagnosis of monoclonal gammopathy of undetermined significance by our hematologist/oncologist. Paraproteinemic keratopathy is a rare differential diagnosis in patients with bilateral corneal opacities and therefore may be misdiagnosed as corneal dystrophy or neglected as scars. In patients with bilateral corneal opacities of unknown cause, serological examination, adjunct anterior segment imaging, and molecular genetic testing play a role in establishing the diagnosis.

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Section: Discussionmentioning

confidence: 99%

Diagnosing Paraproteinemic Keratopathy: A Case Report

Mok

Kam

Aldave

et al. 2021

Case Rep Ophthalmol

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“…Given the wide array of corneal deposition patterns, ophthalmologists should consider bilateral ocular findings as possible manifestations of systemic disease, necessitating multidisciplinary investigation for diagnostic confirmation. 9,10 This patient had no known history of blood dyscrasia, yet the corneal findings led to the discovery of underlying MGUS.…”

Section: Slitlamp Photograph Of Left Eyementioning

confidence: 99%

Bilateral Asymmetrical Corneal Deposits in an Asymptomatic Patient

2021

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A 70-year-old White woman with a history of hypertension and hyperlipidemia was referred to our institution for evaluation of bilateral asymmetric corneal lesions by her primary eye care professional. The patient required spectacles for best-corrected visual acuity but otherwise had no other significant ocular complaints. She was not taking medications known to cause corneal depositions, nor was there a known family history of corneal dystrophy. Best-corrected visual acuity was 20/20 OU. Slitlamp examination of the cornea of the right eye revealed multiple, discrete nodular opacities in the superior and inferior quadrants without neovascularization or surface elevation. Similar findings were seen in the left eye, but the superior lesions were notable for possessing well-circumscribed, plaquelike features located in the posterior stroma without involvement of the anterior surface (Figure , A). No crystalline changes were noted with these deposits.For further characterization of the corneal findings, anterior segment optical coherence tomography (AS-OCT) was obtained, revealing multiple anterior stromal and subepithelial opacities without involvement of the deeper stromal layers in the right eye. AS-OCT of the left eye showed multiple anterior and posterior stromal opacities with sparing of the central stromal layers (Figure , B). While the posterior lesions caused a bulging of Descemet membrane, the endothelial layer was intact in both eyes.

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“…They can appear as one of the extramedullary manifestations of the disease or as the first sign of an ineffective therapy [ 9 , 10 ]. PPK can be associated with lymphoma, leukemia, cryoglobulinemia, rheumatoid arthritis or Waldenström macroglobulinemia [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…The corneal stroma appears to be the most frequently affected corneal layer, followed by the epithelium and the subepithelium [ 11 – 13 ]. Endothelium and Descemet’s membrane are rare locations of the paraprotein deposition [ 11 , 14 ]. Crystalline paraprotein deposits may be visualized in the cornea using in vivo confocal laser scanning microscopy (IVCM) [ 15 ].…”

Section: Introductionmentioning

confidence: 99%

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Prevalence of corneal findings and their interrelation with hematological findings in monoclonal gammopathy

Hariri

Munder²,

Lisch³

et al. 2022

PLoS ONE

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Purpose To determine prevalence of paraproteinemic keratopathy (PPK) among patients with monoclonal gammopathy (MG). To evaluate interrelation between corneal and hematological parameters in patients with PPK. Methods Fifty-one patients with monoclonal gammopathy of undetermined significance (n = 19), smoldering multiple myeloma (n = 5) or multiple myeloma (n = 27) were prospectively included in this study. Best-corrected visual acuity, slit-lamp biomicroscopy, Scheimpflug tomography, in-vivo confocal laser scanning microscopy, optical coherence tomography and complete hematological workup were assessed. Results We identified n = 19 patients with bilateral corneal opacities compatible with PPK. PPK was newly diagnosed in 13 (29%) of 45 patients with a primary hematological diagnosis and in n = 6 patients without previous hematological diagnosis. The most common form was a discreet stromal flake-like PPK (n = 14 of 19). The median level of M-protein (p = 0.59), IgA (p = 0.53), IgG (p = 0.79) and IgM (p = 0.59) did not differ significantly between the patients with and without PPK. The median level of the FLC κ in serum of patients with kappa-restricted plasma cell dyscrasia was 209 mg/l in patients with PPK compared to 38.1 mg/l in patients without PPK (p = 0.18). Median level of FLC lambda in serum of patients with lambda-restricted plasma cell dyscrasia was lower in patients with PPK compared to patients without PPK (p = 0.02). Conclusion The PPK was mostly discreet, but its prevalence (29%) was higher than expected. Median level of the monoclonal paraprotein was not significantly higher in patients with PPK compared to patients without PPK. Our results suggest a lack of correlation between morphology and severity of the ocular findings and severity of the monoclonal gammopathy. Trial registration German Clinical Trial Register: DRKS00023893.

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Characterization of corneal deposition keratopathy in the setting of blood cell dyscrasia and a minimally invasive technique to clear the cornea in a single case

Cited by 8 publications

References 15 publications

Diagnosing Paraproteinemic Keratopathy: A Case Report

Diagnosing Paraproteinemic Keratopathy: A Case Report

Bilateral Asymmetrical Corneal Deposits in an Asymptomatic Patient

Prevalence of corneal findings and their interrelation with hematological findings in monoclonal gammopathy

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