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Corneal Crystalline Deposits Associated With Dysproteinemia: Title and subTitle BreakReport of Two Cases and Review of the Literature FREE

Charles C. Barr, MD; Henry Gelender, MD; Ramon L. Font, MD
[+] Author Affiliations

Accepted for publication Oct 1, 1979.

Reprint requests to Bascom Palmer Eye Institute, PO Box 016880, Miami, FL 33101 (Dr Gelender).


Arch Ophthalmol. 1980;98(5):884-889. doi:10.1001/archopht.1980.01020030878015
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• Two patients had multiple, fine, polychromatic corneal crystals on ocular examination. Peripheral corneal biopsies and electron microscopic studies demonstrated that the crystals, which were located solely within the cytoplasm of keratocytes, exhibited an internal periodicity of 10 nm and developed within dilated cisternae of rough-surfaced endoplasmic reticulum. Histochemical studies of the corneal crystals confirmed their proteinaceous nature (immunoglobulin crystals). Serum protein analysis showed that both patients had a monoclonal gammopathy with an elevated IgG level and increased k light chains. The associated proteinopathy was unsuspected in both patients until the nature of the crystals was established. Both patients had a neoplastic process; one had a previous diagnosis of Hodgkin's disease, and multiple myeloma developed in the other on follow-up studies. Clinicians should be aware of the possibility of a malignant disorder in patients with corneal crystalline deposits.

REFERENCES

Burki E:  Über Hornhautveränderungen bei unem Fall von multiplem Myelom (Plastocytom) . Ophthalmologica 135:565-572, 1958;.
Aronson SB, Shaw R:  Corneal crystals in multiple myeloma . Arch Ophthalmol 61:541-546, 1959;.
Francois J:  Paraproteinaemic thesaurismosis of the cornea in Kohlers' multiple myelomatosis . Eye Ear Nose Throat Monthly 46:857-867, 1967;.
Laibson PR, Damiano VV:  X-ray and electron diffraction of ocular and bone marrow crystals in paraproteinemia . Science 163:581-583, 1969;.
Pinkerton RMH, Robertson DM:  Corneal and conjunctival changes in dysproteinemia . Invest Ophthalmol Vis Sci 8:357-364, 1969;.
Klintworth GK, Bredehoeft SJ, Reed JW:  Analysis of corneal crystalline deposits in multiple myeloma . Am J Ophthalmol 86:303-313, 1978;.
Rodrigues MM, Krachmer JH, Miller SD, et al:  Posterior corneal crystalline deposits in benign monoclonal gammopathy: A clinicopathologic case report . Arch Ophthalmol 97:124-128, 1979;.
Taylor CR:  Immunoperoxidase techniques . Arch Pathol Lab Med 102:113-121, 1978;.
Miale JB: Laboratory Medicine Hematology , ed 4. St Louis, CV Mosby Co, 1972;, 1208-1210.
Kyle RA, Greipp PR:  Laboratory investigation of monoclonal gammopathies . Mayo Clin Proc 53:719-739, 1978;.
Ashton N:  Ocular changes in multiple myelomatosis . Arch Ophthalmol 73:487-494, 1965;.
Johnson BL, Storey JD:  Proteinaceous cysts of the ciliary epithelium: I. Their clear nature and immunoelectrophoretic analysis in a case of multiple myeloma . Arch Ophthalmol 84:166-170, 1970;.
Carr RE, Henkind P:  Retinal findings associated with serum hyperviscosity . Am J Ophthalmol 56:23-31, 1963;.
Gudas PP:  Optic nerve myeloma . Am J Ophthalmol 71:1085-1089, 1971;.
Rodman HI, Font RL:  Orbital involvements in multiple myeloma: Review of the literature and report of three cases . Arch Ophthalmol 87:30-35, 1972;.
Lewis RA, Falls HF, Troyer DO:  Ocular manifestations of hypercupremia associated with multiple myeloma . Arch Ophthalmol 93:1050-1053, 1975;.
Oglesby R:  Corneal opacities in a patient with cryoglobulinemia and reticulohistiocytosis . Arch Ophthalmol 65:63-66, 1961;.
Azar HA, Zaino EC, Pham TD, et al:  "Nonsecretory" plasma cell myeloma observations on severe cases with electron microscopic studies . Am J Clin Pathol 58:618, 1972;.
Bessis MC:  Ultrastructure of lymphoid and plasma cells in relation to globulin and antibody formation . Lab Invest 10:1040, 1961;.
Ito S, Goshima K, Niiomi M, et al:  Electron microscopic studies of the crystalline inclusions in the myeloma cells and kidney of K-Bence-Jones protein type myeloma . Acta Hematol Jap 33:598, 1970;.
Alexanian R:  Monoclonal gammopathy in lymphoma . Arch Intern Med 135:62-66, 1975;.

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Burki E:  Über Hornhautveränderungen bei unem Fall von multiplem Myelom (Plastocytom) . Ophthalmologica 135:565-572, 1958;.
Aronson SB, Shaw R:  Corneal crystals in multiple myeloma . Arch Ophthalmol 61:541-546, 1959;.
Francois J:  Paraproteinaemic thesaurismosis of the cornea in Kohlers' multiple myelomatosis . Eye Ear Nose Throat Monthly 46:857-867, 1967;.
Laibson PR, Damiano VV:  X-ray and electron diffraction of ocular and bone marrow crystals in paraproteinemia . Science 163:581-583, 1969;.
Pinkerton RMH, Robertson DM:  Corneal and conjunctival changes in dysproteinemia . Invest Ophthalmol Vis Sci 8:357-364, 1969;.
Klintworth GK, Bredehoeft SJ, Reed JW:  Analysis of corneal crystalline deposits in multiple myeloma . Am J Ophthalmol 86:303-313, 1978;.
Rodrigues MM, Krachmer JH, Miller SD, et al:  Posterior corneal crystalline deposits in benign monoclonal gammopathy: A clinicopathologic case report . Arch Ophthalmol 97:124-128, 1979;.
Taylor CR:  Immunoperoxidase techniques . Arch Pathol Lab Med 102:113-121, 1978;.
Miale JB: Laboratory Medicine Hematology , ed 4. St Louis, CV Mosby Co, 1972;, 1208-1210.
Kyle RA, Greipp PR:  Laboratory investigation of monoclonal gammopathies . Mayo Clin Proc 53:719-739, 1978;.
Ashton N:  Ocular changes in multiple myelomatosis . Arch Ophthalmol 73:487-494, 1965;.
Johnson BL, Storey JD:  Proteinaceous cysts of the ciliary epithelium: I. Their clear nature and immunoelectrophoretic analysis in a case of multiple myeloma . Arch Ophthalmol 84:166-170, 1970;.
Carr RE, Henkind P:  Retinal findings associated with serum hyperviscosity . Am J Ophthalmol 56:23-31, 1963;.
Gudas PP:  Optic nerve myeloma . Am J Ophthalmol 71:1085-1089, 1971;.
Rodman HI, Font RL:  Orbital involvements in multiple myeloma: Review of the literature and report of three cases . Arch Ophthalmol 87:30-35, 1972;.
Lewis RA, Falls HF, Troyer DO:  Ocular manifestations of hypercupremia associated with multiple myeloma . Arch Ophthalmol 93:1050-1053, 1975;.
Oglesby R:  Corneal opacities in a patient with cryoglobulinemia and reticulohistiocytosis . Arch Ophthalmol 65:63-66, 1961;.
Azar HA, Zaino EC, Pham TD, et al:  "Nonsecretory" plasma cell myeloma observations on severe cases with electron microscopic studies . Am J Clin Pathol 58:618, 1972;.
Bessis MC:  Ultrastructure of lymphoid and plasma cells in relation to globulin and antibody formation . Lab Invest 10:1040, 1961;.
Ito S, Goshima K, Niiomi M, et al:  Electron microscopic studies of the crystalline inclusions in the myeloma cells and kidney of K-Bence-Jones protein type myeloma . Acta Hematol Jap 33:598, 1970;.
Alexanian R:  Monoclonal gammopathy in lymphoma . Arch Intern Med 135:62-66, 1975;.

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