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Kahler disease (myelomatosis; multiple myeloma)


Kahler Disease (Myelomatosis; Multiple Myeloma)

General: Disseminated malignancy of plasma cells located predominantly in the bone marrow.

Ocular: Tumor of orbit common, with proptosis or displacement of globe; conjunctival sledging and segmentation; crystalline deposits of cornea and conjunctiva; cotton-wool spots; retrobulbar neuritis; occlusion of central retinal artery and vein; palsy of sixth nerve; vitreous hemorrhage; dilated veins and hemorrhages; retinal microaneurysms; choroidal detachment; amaurosis fugax; myeloma infiltrates in orbit, iris, choroid, retina, sclera, and optic nerve; corneal opacities; ciliary body cysts; iritis; glaucoma; subluxation of lens; papilledema; corneal edema; cavernous sinus syndrome; bilateral superficial punctate keratitis; central retinal vein occlusion; crystalline keratopathy in a vortex distribution; spontaneous endocapsular hematoma.

Clinical: Bone pain; fractures; dehydration; hypercalcemia; hyperuricemia; proteinuria; inclination to infection; hyperviscosity.

Auran JD, et al. Multiple myeloma presenting as vortex crystalline keratopathy and complicated by endocapsular hematoma. Cornea 1992; 11:584-585.

Hayasaka S, et al. Central retinal vein occlusion in two patients with immunoglobulin G multiple myeloma associated with blood hyperviscosity. Ann Ophthalmol 1993; 25: 191-l94.

Klintworth GK, et al. Analysis of corneal crystalline deposits in multiple myeloma. Am J Ophthalmol 1978; 86:303-313.

Lam S, et al. Cavernous sinus syndrome as the initial manifestation of multiple myeloma. J Clin Neuro-Ophthalmol 1987; 7:135.

Lee H, et al. Periorbital ecchymoses as the initial sign in multiple myeloma. Ann Ophthalmol 1982; 14:1066-l068.

Maisel JM, et al. Multiple myeloma presenting with ocular inflammation. Ann Ophthalmol 1987; 19:170-l74.

Mewis-Levin L, et al. Plasma cell myeloma of the orbit. Ann Ophthalmol 1981; 13:477-481.

Perry HD, et al. Intraepithelial corneal immunoglobulin crystals in IgG-kappa multiple myeloma. Cornea 1993; 12:448-450.




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