1. Josephine-Liezl Cueto, M.D.* Kendall R. Dobbins, M.D.* Geisinger Medical Center, Department of Ophthalmology Danville, PA *No financial interest

2. Purpose To report a case of asymptomatic bilateral chronic anterior uveitis unresponsive to topical steroids in a patient with a history of chronic lymphocytic leukemia (CLL). While the consensus was that masquerade syndrome was high on the differential diagnosis, we describe how aqueous fluid analysis was crucial in determining that it was not the final diagnosis. 2

3. Methods 65 year old male with a history of CLL who presents with decreased vision in both eyes at distance and near He was referred by an outside Ophthalmologist for a 3 month history of bilateral chronic uveitis that was unresponsive to high dose topical steroid treatment PMH: CLL treated with chemotherapy POH: None ROS: no eye pain, no photophobia, no rash, no gastrointestinal problems 3

4. Methods Va: OD: 20/60 PH 20/20- OS: 20/100 PH NI Pupils No RAPD IOP Ta: 13 OD, 14 OS Cornea WBC’s coating endothelium A/C 3-4+ cell and flare OU Lens: 2+NSC and 1+PSC OU Fundus Exam: Vitreous Clear OU ON 0.1 OU Retina Normal OU 4

5. Methods Work up All negative: RPR, FTA-ABS, Anti-nuclear Antibody, ACE, HLA B27 After one month of increasing the frequency of topical steroid treatment, the patient’s clinical exam remained unchanged Since the patient lacked any ocular inflammatory symptoms and had a negative serum inflammatory evaluation, the leading diagnosis was masquerade syndrome secondary to CLL To confirm this, an anterior chamber tap was performed 5

6. Results Anterior Chamber Tap cytology: A monomorphic population of lymphocytes No B-cell clonal population Since CLL is a B-cell process and the A/C tap only revealed T-cells, the results indicated either an inflammatory response or a new lymphoproliferative process 6

7. Results To rule out a new lymphoproliferative process, a peripheral smear was done Findings: Consistent with CLL No new T-cell process 7

8. Results Since the peripheral smear revealed CLL and no new peripheral lymphoproliferative disorder, the ophthalmic process was confirmed to be inflammatory 8

9. Results Since the anterior chamber tap and peripheral smear proved the ocular process to be inflammatory, a subtenons steroid injection and oral steroids were added to the treatment regimen of very high dose topical steroids 9

10. Results After three months of treatment, the anterior chamber inflammatory process was almost completely resolved and the oral steroids were tapered After eight months of treatment, the topical steroids were tapered with resolvement of the anterior uveitis 10

11. Conclusion In this atypical case of uveitis, while clinical suspicion was very high for masquerade syndrome, flow cytometry revealed the case to be an extremely unusual inflammatory process This unique case demonstrates the usefulness of diagnostic analysis of aqueous in case of presumed uveitis refractory to standard treatment While there are only two reported cases 1,2 of hematologic cancers diagnosed by an anterior chamber tap, this is the only reported case where a highly suspected masquerade syndrome secondary to a previously diagnosed blood dyscrasia was proven to be an inflammatory process by anterior chamber analysis 11

12. References 1. Birnbaum AD, Tessler HH, Goldstein DA. A Case of Hypopyon Uveitis Nonresponsive to Steroid Therapy and a Review of Anterior Segment Masquerade Syndromes in Childhood. J Pediatr Ophthalmol Strabismus 2005;42:372-377. 2. Verbraeken HE, Hanssens M, Hildegaard P, et al. Br J Ophthalmol 1997; 81:31-36. 12