Download Asymptomatic Unresponsive Bilateral Uveitis Posing as a

Josephine-Liezl Cueto, M.D.*
Kendall R. Dobbins, M.D.*
Geisinger Medical Center, Department of Ophthalmology
Danville, PA
*No financial interest
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.
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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
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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
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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
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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
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Results
 To rule out a new
lymphoproliferative
process, a peripheral
smear was done
 Findings:
 Consistent with CLL
 No new T-cell process
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Results
 Since the peripheral smear revealed
CLL and no new peripheral
lymphoproliferative disorder, the
ophthalmic process was confirmed to
be inflammatory
8
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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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
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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
11
References
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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