Asymptomatic Unresponsive Bilateral Uveitis Posing as a Masquerade Syndrome in a Patient with Chronic Lymphocytic Leukemia

1
Asymptomatic Unresponsive Bilateral Uveitis
Posing as a Masquerade Syndrome in a Patient with
Chronic Lymphocytic Leukemia

• Josephine-Liezl Cueto, M.D.
• Kendall R. Dobbins, M.D.
• Geisinger Medical Center, Department of
  Ophthalmology
• Danville, PA

No financial interest
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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.

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

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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
• WBCs coating endothelium

• A/C
• 3-4 cell and flare OU
• Lens
• 2NSC and 1PSC 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 patients 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

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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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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

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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 200542372-377.
2. Verbraeken HE, Hanssens M, Hildegaard P, et al.
   Br J Ophthalmol 1997 8131-36.