Immunoglobulin G crystalline keratopathy associated with monoclonal gammopathy

Hutchinson, Kenneth; Pra, Mark Dal; Apel, Andrew

doi:10.1111/j.1442-9071.1998.tb01539.x

Cited by 11 publications

(6 citation statements)

References 4 publications

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“…Corneal crystalline deposits have been seen in patients with MGUS (227–230). Monoclonal gammopathies have been reported in many different conditions such as Hashimoto's thyroiditis, purpura fulminans, Schönlein‐Henoch purpura, septic arthritis, idiopathic pulmonary fibrosis, pulmonary alveolar proteinosis, idiopathic pulmonary hemosiderosis, sarcoidosis, thymoma, hereditary spherocytosis, and hyperlipoproteinemia.…”

Section: Association Of Monoclonal Gammopathy With Other Diseasesmentioning

confidence: 99%

Monoclonal gammopathies of undetermined significance: a review

Kyle

Rajkumar

2003

Immunological Reviews

109

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Monoclonal gammopathy of undetermined significance (MGUS) denotes the presence of a monoclonal protein (M-protein) in patients without evidence of multiple myeloma (MM), macroglobulinemia, amyloidosis (AL), or a related plasma cell proliferative disorder. MGUS is found in approximately 3% of persons older than 70 years and in about 1% of those older than 50 years. In a series of 1384 patients from south-eastern Minnesota in whom MGUS was diagnosed at Mayo Clinic from 1960 through 1994, the risk of progression was 1% per year. Patients were at risk of progression even after 25 years or more of a stable monoclonal gammopathy. The risk of development of MM was increased by 25-fold, the risk of macroglobulinemia was 46-fold, and the risk of primary AL was 8.4-fold when compared with a similar population (Surveillance, Epidemiology and End Results). The concentration of the serum M-protein was the major independent predictor of progression. Patients with an immunoglobulin M (IgM) or an IgA monoclonal gammopathy had a higher risk of progression than those with an IgG monoclonal gammopathy. The presence of a urine M-protein or the reduction of one or more uninvolved Igs was not a risk factor for progression. MGUS may be associated with many different disorders, including lymphoproliferative diseases, leukemia, connective tissue disorders, dermatologic diseases, and neurologic disorders.

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Section: Association Of Monoclonal Gammopathy With Other Diseasesmentioning

confidence: 99%

Monoclonal gammopathies of undetermined significance: a review

Kyle

Rajkumar

2003

Immunological Reviews

109

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“…Corneal epithelial disturbance in a spiraling pattern has been rarely reported in patients with dysproteinemias such as MM and SMM9, 10, 11, 12, 13; in these patients corneal features more frequently include stromal haze, lattice and crystalline dystrophy, secondary to immunoglobulin deposition in the various corneal layers 1 , 11 …”

Section: Discussionmentioning

confidence: 99%

“…The presence of diffuse granular and spindle-shaped crystals (both intracellular and extracellular) at the level of the corneal epithelium and stroma (crystalline keratopathy) has been reported in patients with MG3, 4, 5 and in those with multiple myeloma (MM) 6, 7, 8. While deposits representing a vortex keratopathy (cornea verticillata-like PPK) has been described in four patients with MM9, 10, 11, 12 and in one patient with MGUS, 13 in none of these prior reports had a confocal microscopy evaluation been performed.…”

Section: Introductionmentioning

confidence: 99%

In vivo confocal microscopy of verticillata-like paraproteinemic keratopathy in a patient with monoclonal gammopathy of uncertain significance evolving into smoldering multiple myeloma

Chierego

Merz

Fasolo

et al. 2019

American Journal of Ophthalmology Case Reports

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Purpose To highlight the utility of in vivo confocal microscopy (IVCM) in the microstructural characterization of corneal deposits resembling vortex keratopathy in a case of secondary deposition keratopathy due to an evolving monoclonal gammopathy. Observations A 56-year-old Caucasian woman previously diagnosed with monoclonal gammopathy of undetermined significance (MGUS), exhibited bilateral diffuse sub-epithelial and anterior stromal opacities distributed in a whorl-shaped pattern. IVCM examination permitted analysis of the microstructural features and distribution of the opacities in different corneal layers. Deposits resembling those reported in multiple myeloma rather than MGUS were detected, and the patient's hematologist was subsequently advised to re-evaluate the earlier diagnosis. Two months later, a bone marrow biopsy led to a diagnosis of plasma cell myeloma. Conclusions and importance It is suggested that cornea verticillata-like paraproteinemic keratopathy (PPK) in MGUS might be predictive of disease evolution to plasma cell myeloma. Characteristic deposits and morphological features revealed by IVCM may be helpful for non-invasive assessment or screening in patients with hematological disorders.

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“…5 One study showed that 19% of 241 patients with a diagnosis of benign monoclonal gammopathy developed myeloma, or a related disorder when followed for 10 years or more. 6 Morphological changes of the corneal crystals were noted previously in a patient of IgG-kappa monoclonal gammopathy.…”

Section: Discussionmentioning

confidence: 99%