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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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