Paraproteinemic Corneal Deposits in Plasma Cell Myeloma

Steuhl, Klaus‐Peter; Knorr, M.; Rohrbach, Jens Martin; Lisch, Walter; Kaiserling, E.; Thiel, Holger

doi:10.1016/s0002-9394(14)72315-3

Cited by 29 publications

(15 citation statements)

References 20 publications

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“…One theory is that the deposits result from elevated Ig levels in the tears and aqueous humor;20 another is that they enter the cornea from the limbal blood vessels; and a third is that the keratocytes themselves synthesize the precipitated Ig. Henderson et al21 discuss that some of the reasons for Ig deposition are: 1) an inherent propensity of some Ig or kappa light chains to crystallize; and 2) local factors in the cornea that may promote deposition, such as temperature, pH, water content, and extracellular matrix deposition.…”

Section: Discussionmentioning

confidence: 99%

Cloudy corneas as an initial presentation of multiple myeloma

Sharma

Madi

Bonshek

et al. 2014

OPTH

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SummaryWe report a case of previously unsuspected myeloma, presenting with cornea verticillata due to intracorneal paraprotein deposition.HistoryAn 85-year-old female presented via her optician with a 4-month history of cloudy vision. She had undergone an uneventful bilateral phacoemulsification surgery 7 years earlier. Extensive spiraling corneal epithelial opacification was noted on slit-lamp examination. On further investigation, she was found to have a previously unsuspected low-grade multiple myeloma. We established the nature of the corneal deposits with corneal epithelial biopsy histopathology and electron microscopy. It is very rare for multiple myeloma to present in this fashion. Ophthalmologists should be aware that such a presentation may rarely be due to systemic multiple myeloma.

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Section: Discussionmentioning

confidence: 99%

Cloudy corneas as an initial presentation of multiple myeloma

Sharma

Madi

Bonshek

et al. 2014

OPTH

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“…Steuhl et al . [7] and Cherry et al . [2] had demonstrated the presence of Igs in the ocular tears of an IgG-kappa monoclonal gammopathy patient who had deposits limited to the corneal epithelium.…”

Section: Discussionmentioning

confidence: 99%

“…There are only scattered reports describing endothelial involvement; in the majority of biopsy proven cases, the deposits have been found to be localized to the epithelium and or stroma. [79] It may be difficult to differentiate direct endothelial from predescemetic localization using light to electron microscopy and IVCM may contribute in identifying endothelial involvement in paraproteinemias. [10]…”

Section: Discussionmentioning

confidence: 99%

In vivo confocal microscopic characteristics of crystalline keratopathy in patients with monoclonal gammopathy: Report of two cases

Kocabeyoğlu

Mocan

Haznedaroğlu

et al. 2014

Indian J Ophthalmol

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In this paper, we report two cases of a 62-year-old patient presented with blurred vision and a 45-year-old male diagnosed with multiple myeloma who was referred from the Department of Oncology. Slit-lamp examination, in vivo confocal microscopy (IVCM), systemic work-up and serum protein electrophoresis were obtained. In both patients, slit-lamp findings revealed bilateral diffuse subepithelial and anterior stromal crystals and IVCM showed highly reflective deposits in the corneal epithelium and stroma. The first patient was eventually diagnosed with monoclonal gammopathy of undetermined significance following bone marrow biopsy and systemic evaluation. Unusual corneal deposits may constitute the first sign of monoclonal gammopathies. IVCM may be helpful in showing the crystalline nature of the corneal deposits and guiding the clinician to the diagnosis of gammopathies. Both ophthalmologists and oncologists should be aware that corneal deposits may herald a life-threatening hematologic disease.

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“…7 In fact, in gammopathies not accompanied by general symptoms and signs, the diagnosis of primary corneal diseases, such as lattice dystrophy, 8,9 Groenouw type I granular dystrophy, 10 or deep filiform dystrophy, 11 or the diagnosis of systemic diseases such as cystinosis, 12,13 was established. 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.…”

mentioning

confidence: 99%

“…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.…”

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confidence: 99%

Corneal Structural Changes in Nonneoplastic and Neoplastic Monoclonal Gammopathies

Aragona

Allegra

Postorino

et al. 2016

Invest. Ophthalmol. Vis. Sci.

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METHODS. Three groups of subjects were considered: group 1, twenty normal subjects; group 2, fifteen patients with monoclonal gammopathy of undetermined significance (MGUS); group 3, eight patients with smoldering multiple myeloma and eight patients with untreated multiple myeloma. After hematologic diagnosis, patients underwent ophthalmologic exam and in vivo confocal microscopic study. The statistical analysis was performed using ANOVA and Student-Newman-Keuls tests and receiver operating characteristic (ROC) curve analysis.RESULTS. Epithelial cells of gammopathic patients showed significantly higher reflectivity than controls, demonstrated by optical density (P < 0.001). Subbasal nerve density, branching, and beading were significantly altered in gammopathic patients (P ¼ 0.01, P ¼ 0.02, P ¼ 0.02, respectively). The number of keratocytes was significantly reduced in neoplastic patients (P < 0.001 versus both normal and MGUS) in the anterior, medium, and posterior stroma. The ROC curve analysis showed good sensitivity and specificity for this parameter. Group 2 and 3 keratocytes showed higher nuclear and cytoplasmatic reflectivity in the medium and posterior stroma. Endothelial cells were not affected. CONCLUSIONS.Patients with neoplastic gammopathies showed peculiar alterations of the keratocyte number, which appeared significantly reduced. A follow-up with corneal confocal microscopy of patients with MGUS is suggested as a useful tool to identify peripheral tissue alterations linked to possible neoplastic disease development.

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Paraproteinemic Corneal Deposits in Plasma Cell Myeloma

Cited by 29 publications

References 20 publications

Cloudy corneas as an initial presentation of multiple myeloma

Cloudy corneas as an initial presentation of multiple myeloma

In vivo confocal microscopic characteristics of crystalline keratopathy in patients with monoclonal gammopathy: Report of two cases

Corneal Structural Changes in Nonneoplastic and Neoplastic Monoclonal Gammopathies

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