Table 1.

Cases of Ocular Pathology Associated with Essential Monoclonal Gammopathy.

Citation/Year of Report Age Sex (M/F) Ig Isotype Other Tissue Involvement Treatment Outcome Notes
Crystalline Keratopathy
12/1959 55 F IgG (light chain type not determined) Iritis, conjunctivitis None at the time of diagnosis Five years after diagnosis of keratopathy, myeloma developed with crystals in myeloma cells
7/1979 74 F IgG-kappa No Keratoplasty and cataract extraction Not reported Posterior corneal stroma contained parallel structures with periodicity of 10–11 nm
17/1980 57 M IgG-kappa Iritis Not reported Not reported History of Hodgkin lymphoma treated with radiation
14/1980 39 F IgG-kappa Tearing, photophobia Not reported Not reported IgGk deposits in corneal stroma and Bowman membrane. Electron micrograph showed parallel rod-like structures with periodicity of 10 nm
23/1985 65 F IgA-kappa Macular drusen Not reported Not reported Slit lamp examination showed needle-like refractile, coarse crystals in mid-deep corneal stroma, bilaterally
16/1988 50 F IgG-kappa Uveitis Corneal graft and posterior chamber intraocular lens insertion Recurrence of crystalline keratopathy by 4 months TEM revealed paracrystals with periodicity of 16 nm
25/1989 60 M IgG-kappa (essential cryo-immunoglobulin) No Superficial keratectomy Improved visual acuity Crystallization at the corneal subepithelium in this case thought to be related to that site being the coldest in the body (~32°C)
28/1993 62 F IgG-kappa No Not reported Not reported Crystal deposits by TEM were all extracellular, located between keratocytes
28/1993 65 M IgG-kappa No Not reported Not reported Majority of crystals by TEM were intracellular in stromal keratocytes
29/1994 62 M IgG-kappa No Penetrating keratoplasty Not reported IgGk deposits in cornea; TEM showed intracytoplasmic Ig in keratocytes with 10 nm periodicity
20/1996 52 F IgG-kappa Photophobia and foreign body sensation Not reported Not reported TEM of corneal biopsy found extracellular rectangular and arcuate crystalloids with 10 nm periodicity
19/1999 55 F IgG-kappa No Penetrating keratoplasty, open-sky extracapsular cataract extraction, posterior chamber intraocular lens implantation Not reported Decreasing visual acuity led to diagnosis crystalline keratopathy and, subsequently, of IgG-kappa essential monoclonal gammopathy. IgG-kappa-positive deposits in corneal button removed at keratoplasty; TEM showed numerous bundles of electron dense deposits of filaments with a periodicity of 10–13 nm in the anterior stroma of the cornea. Some deposits were phagocytosed by keratocytes
18/2011 62 F IgG-kappa No Three penetrating keratoplasty surgeries, right-sided: at diagnosis, then 9 years and 12 years later for recurrent loss of visual acuity in the right eye Improvement for several months post-keratoplasty followed by gradual decrease in visual acuity over years Left eye affected but to a lesser degree than right eye; no follow-up reported after 3rd keratoplasty
Crystal-storing Histiocytosis Involving the Eye
35/1990 64 F IgA-kappa No Not described Not described Slit lamp exam revealed bilateral superficial corneal and conjunctival crystals. Diagnosed by conjunctival biopsy
10/1993 62 F IgG-kappa Crystalline keratopathy 4 cycles of bortezomib-based chemotherapy followed by autologous stem cell transplantation 18 months following autologous stem cell transplantation, patient in complete hematologic, renal, and ophthalmologic remission Melphalan conditioning for auto-transplant. At 3 months post-transplant, orbital mass had significantly decreased, Fanconi syndrome resolved, and there was no detectable paraproteinemia
34/2009 66 M IgG-lambda Crystalline keratopathy Not described Not described Right-sided keratoplasty for crystalline keratopathy 1 year prior to diagnosis of CSH. MRI revealed enhancement of retrobulbar fat and thickening of extraocular muscles; biopsy revealed CSH
Corneal Deposition of Immunoglobulin-bound Copper
42/1967 69 F IgG No Not described Not described Myeloma diagnosed 1 year after slit lamp findings of greenish-blue granular discoloration of corneal epithelium and Descemet membrane. Serum copper 13 to 24 × values found in 11 other unaffected myeloma patients tested. Serum ceruloplasmin was normal. Mild increase in urinary copper
44/1975 41 F IgG-lambda No Not described Not described 3 years before diagnosis of myeloma, patient had diagnosis of “cataracts” despite 20/20 vision. Soon thereafter cornea found to have dense brown staining reminiscent of Kayser–Fleischer type corneal rings. Serum copper 7 to 11 × normal serum value. Serum ceruloplasmin normal
39/1983 60 M IgG-lambda Not reported Intracapsular cataract extraction and insertion of Binkhorst four-loop lens Not reported Concurrent diagnosis of lung carcinoma. Received radiation to lung. Slit lamp examination showed diffuse greenish-yellow discoloration of cornea. Serum copper 7 × normal serum value. Serum ceruloplasmin normal
41/1996 65 M IgG-kappa Copper depositions in anterior and posterior lens surface and appearance of sunflower cataract Zinc gluconate No change in serum copper Bilateral blurred vision. Slit lamp examination revealed golden-brown deposits diffusely distributed centrally in cornea. Serum copper 12 × normal serum value. Serum ceruloplasmin normal. Special studies showed binding of copper to serum monoclonal IgG by two methods
40/2005 49 F IgG-lambda No No N/a Patient asymptomatic. Slit lamp examination showed golden-brown metallic dust-like particles in Descemet membrane bilaterally. Serum copper 3 × normal. Serum ceruloplasmin normal
38/2014 46 F IgG-lambda No Descemet-stripping endothelial keratoplasty and cataract extraction with intraocular lens implant of left eye Complete return of normal visual acuity (20/20) Posterior layer of both corneas had a confluent tan color with only a narrow rim of clear cornea peripherally. Serum copper 12 × normal. Serum ceruloplasmin normal
47/2013 37 F Not specified No Prednisone with inability to wean until rituximab was initiated Marked and rapid improvement in inflammation and visual acuity with prednisone but recurrence with taper. Resolution of ocular findings without recurrence at 9 months once rituximab therapy was initiated Bilateral serous macular detachments, iritis, vitritis. Details of how frequently and at what dose rituximab treatment and maintenance treatment were administered are not provided

CSH, crystal-storing histiocytosis; F, female; M, male; MRI, magnetic resonance imaging; TEM, transmission electron microscopy.

RMMJ Rambam Maimonides Medical Journal Rambam Health Care Campus 2015 July; 6(3): e0026. ISSN: 2076-9172
Published online 2015 July 30. doi: 10.5041/RMMJ.10211