Josephine-Liezl Cueto, M.D.*Kendall R. Dobbins, M.D.*

Geisinger Medical Center, Department of OphthalmologyDanville, PA

*No financial interest

PurposeTo 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.

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Methods65 year old male with a history of CLL who

presents with decreased vision in both eyes at distance and nearHe 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 chemotherapyPOH: NoneROS: no eye pain, no photophobia, no rash, no

gastrointestinal problems3

MethodsVa:

OD: 20/60 PH 20/20-

OS: 20/100 PH NIPupils

No RAPDIOP

Ta: 13 OD, 14 OSCornea

WBC’s coating endothelium

A/C3-4+ cell and flare OU

Lens:2+NSC and 1+PSC

OUFundus Exam:

Vitreous Clear OU

ON 0.1 OU

Retina Normal OU

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MethodsWork 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

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ResultsAnterior 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

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ResultsTo rule out a new

lymphoproliferative process, a peripheral smear was done

Findings:Consistent with CLLNo new T-cell

process

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ResultsSince the peripheral smear revealed CLL and no new peripheral lymphoproliferative disorder, the ophthalmic process was confirmed to be inflammatory

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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

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ResultsAfter 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

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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

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References1. 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.

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