Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities in a 61-year-old man

Case Report A 61-year-old male Transportation driver Vague past medical history of non- symptomatic dysproteinemia Recent decrease in visual acuity

Slit Lamp Biomicroscopy

Case Report VA: OD: 20/160 OS: 20/200 No evidence of conjunctivitis or uveitis IOP: WNL Funduscopy: Unremarkable

Corneal Confocal Scan

Clinical diagnosis of a “Crystalline Keratopathy” Systemic work-up Diagnostic Anterior Lamellar Keratoplasty OS … Case Report

Anterior corneal lamella fixed in absolute alcohol weas sent to our ophthalmic pathology laboratory Gross examination: Two folded translucent pieces of tissue measuring 7x4x1mm and 5x3x1mm; which both were bisected and processed for histopathologic examination … Case Report

Kappa chain

Lambda chain

Histopathological Diagnosis Paraproteinemic Crystalline Keratopathy

Systemic Work-up IgG Monoclonal Gammopathy Free kappa chains in urine Lymphocytosis with 5% plasma cells and a micro and macroblastic reaction on bone marrow biopsy Negative whole body scan Refer to Haematologist Chemotherapy was not decided

Corneal deposits recurred at the deep layers of the donor cornea Repeat confocal scan confirmed recurrence of the crystalline deposits in the graft Refer to haematologist for chemotherapy Four Months after Keratoplasty

Can be classified from the clinical and genetical point of view into 3 groups: 1)Primary hereditary (Schnyder) 2)Secondary hereditary (Cystinosis) 3)Secondary non-hereditary in association with disorders of serum protein or lipid composition Non Infectious Crystalline Keratopathy

In association with disorders of serum protein composition In cases above 50 years of age Bilateral corneal opacities may be the first clinical evidence of systemic disease Paraproteinemic Crystalline Keratopathy

Mechanism of Corneal Crystallization Unknown May be spontaneous Plasma cells containing crystals infiltrate the cornea during episodes of conjunctivitis or anterior uveitis that sometimes precede the occurrence of corneal crystals

Occurs as punctate or linear opacities with an irregular geographic or plaque-like configuration Paraproteinemic Crystalline Keratopathy

Affects the epithelium and the anterior or posterior portion of the stroma Limbal area in most cases is spared Corneal vascularization or loss of corneal sensation has not been reported Recurrence of the immune crystalline deposits in the grafted cornea is common in cases with uncontrolled systemic disorder Paraproteinemic Crystalline Keratopathy

* Confocal Microscopy* As a non invasive diagnostic method is a useful technique for diagnosing and following up such cases before and after systemic therapy and/or corneal transplantation. Paraproteinemic Crystalline Keratopathy

Histopathology Homogeneous eosinophilic and PAS-positive deposits in the epithelium and stroma that stain brilliant red with Masson's trichrome The deposits are strongly immune reactive for IgG-kappa chain and occasionally mildly for lambda chain.

H aematology consultation and proper systemic chemotherapy could be of help T he opacities gradually diminish in patients receiving appropriate therapy O ccasionally keratoplasty is necessitated in addition to the systemic therapy Management

A rare case of “Paraproteinemic Crystalline Keratopathy” as a first clinical evidence of a non-symptomatic hypergammaglobulinemia Recurred after lamellar corneal graft Haematology control would be necessary to control the systemic as well as the ocular disorder.

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