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IgG λ type (stage IIIA) in 2000. He was first managed with vincristine,<br />
adriamycin and dexamethasone, resulting in partial response (PR), and<br />
then with cyclophosphamide and dexamethasone, resulting in complete<br />
response (CR), followed by autologous stem cell transplantation.<br />
No M-component was detected until May 2008, when a solitary<br />
plasmocytoma of the humerus appeared and was managed with<br />
thalidomide. Soon thereafter, a new, diffusely infiltrating unilateral iris<br />
lesion developed.<br />
Results. At diagnosis the patient was asymptomatic with 20/20 vision,<br />
IOP 14 mmHg, and cells in the anterior chamber. The iris was diffusely<br />
reddish with a circle of pigment epithelial cysts around the pupillary<br />
margin. The fundus was normal. He received two courses of bortezomib,<br />
a reversible proteasome inhibitor, with dexamethasone. After the 1st<br />
course, the cells and the redness of the iris disappeared. Two months<br />
after the 2nd course of bortezomib, while on lenalidomide maintenance<br />
therapy, he continued to be asymptomatic with an IOP of 14 mmHg and<br />
a normal iris.<br />
Conclusions. Bortezomib was effective in eradicating an intraocular<br />
replase of multiple myeloma in a patient who previously had received<br />
several types of conventional chemotherapy.<br />
Financial disclosure. None<br />
1923 OTp107<br />
TUBEROUS SCLEROSIS COMPLEX: CHARACTERIZA-<br />
TION OF OCULAR MANIFESTATIONS AND CORRELA-<br />
TIONS WITH SYSTEMIC DISEASE<br />
M.E. Turell1, E.I. Traboulsi1, A. Gupta2 , A.D. Singh1 (turellm2@ccf.org)<br />
1. Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH<br />
2. Department of Pediatric Epilepsy, Cleveland Clinic Foundation,<br />
Cleveland, OH<br />
Purpose. To evaluate genotype/phenotype correlations in individuals<br />
with astrocytic hamartoma (AH) and retinal achromic patch (AP) in the<br />
setting of tuberous sclerosis complex (TSC).<br />
Methods. Participants included 132 patients from the Cleveland Clinic<br />
Foundation Tuberous Sclerosis <strong>Program</strong> (CCF-TSCP) and 907 patients<br />
from the TSC Alliance (TSC-A). Gender, age at TSC diagnosis, presence<br />
of TSC1 or TSC2 mutations, ophthalmic examination, and systemic<br />
manifestations were analyzed.<br />
Results. No difference was found in the prevalence of AH in the CCF-<br />
TSCP (36.1%) and TSC-A (34.1%) groups (p = 0.743). AH were bilateral<br />
in 43.3% and 18.1% (p = 0.009) and multiple in 40.0% and 15.3% (p =<br />
0.008) in the CCF-TSCP and TSC-A groups respectively. In the CCF-TSCP<br />
group, AP was observed in 12.0% of patients (40.0% bilateral, 50.0%<br />
multiple). The presence of retinal features was associated with giant cell<br />
astrocytoma (37.1% vs 14.6%; p = 0.018), renal angiomyolipoma (60.0%<br />
vs 27.1%; p = 0.003), cognitive impairment (77.1% vs 43.8%; p = 0.002),<br />
and epilepsy (91.4% vs 70.8%; p = 0.022) in those with and without<br />
retinal findings respectively. In patients with retinal findings in the CCF-<br />
TSCP and TSC-A groups, mutations in TSC2 were more frequent than<br />
in TSC1, 3.3 times and 5.8 times, respectively. In those without retinal<br />
findings; the relative rates were 0.67 times and 2.3 times, respectively.<br />
Conclusions. Individuals with retinal findings are more likely to have<br />
subependymal giant cell astrocytomas, renal angiomyolipomas,<br />
cognitive impairment and epilepsy. TSC2 mutations are more frequent<br />
in patients with retinal findings than in those without.<br />
Financial disclosure. None<br />
EYELID, CONJUNTIVA & ORBIT<br />
Abstract Posters<br />
88<br />
Support: Research to Prevent Blindness<br />
Challenge Grant, Department of<br />
Ophthalmology, Cleveland Clinic Lerner<br />
College of Medicine<br />
248 OTp108<br />
INTERLEUKIN LEVELS IN AQUEOUS OF UNTREATED<br />
AND TREATED EYES WITH VITREORETINAL LYMPHO-<br />
MA<br />
Jose S. Pulido, MD, Joseph Balsanek, Brian Peters, Melissa Snyder, PhD<br />
(pulido.jose@mayo.edu)<br />
Department of Ophthalmology, Department of Laboratory Medicine and<br />
Pathology<br />
Purpose. To determine the levels of interleukin levels in aqueous<br />
samples taken at the time of intravitreal treatment in patients with<br />
vitreoretinal lymphoma<br />
Methods. Samples at the time of paracentesis were immediately placed<br />
on ice and then ELISA for interleukin levels were performed<br />
Results. 81 samples were tested. IL 10 levels had a mean of 62 with<br />
range of 0.69-624 pg/ml. IL-6 had a mean 123 pg/ml with range from<br />
1.67-960 pg/ml. Other levels that were elevated included were MCP-<br />
1,and IL-1ra.<br />
Conclusions. There are marked changes in interleukin levels depending<br />
upon treatment levels. IL-10 aqueous levels is a good measure of<br />
treatment response to intravitreal chemotherapeutic agents<br />
Financial disclosure. None<br />
304 OTp109<br />
INTRAVITREAL RITUXIMAB FOR PRIMARY INTRAOCU-<br />
LAR LYMPHOMA (PIOL)<br />
V. Kakkassery1, G. Willerding1, K. Jahnke2, A. Korfel2, U. Pleyer1, N.<br />
Stübiger1, A.M. Joussen1 (vk@charite.de)<br />
1. Department of Ophthalmology, Charité, Berlin, Germany<br />
2. Department of Hematology and Oncology, Campus Benjamin Franklin,<br />
Charité, Berlin, Germany<br />
Purpose. PIOL is a rare manifestation of primary central nervous system<br />
lymphoma (PCNSL). Due to its rarity, the optimal treatment of this<br />
condition has not yet been defined thus far. The chimeric monoclonal<br />
CD20 antibody rituximab offers a new intravitreal treatment option for<br />
PIOL. Here, we report on the clinical course after repeated intravitreal<br />
injections of rituximab for PIOL.<br />
Methods. Diagnosis of PIOL was confirmed by clinical investigation<br />
and vitreous biopsy in three cases. Two patients were pretreated with<br />
systemic chemotherapy for their PCNSL (ifosfamide or MTX). Ocular<br />
clinical findings (visual acuity, intraocular pressure, vitreous haze, extent<br />
of tumor) were recorded before and after rituximab therapy. Intravitreal<br />
1mg/0,1mL rituximab injections were conducted in accordance with the<br />
German Ophthalmic Society guidelines for intravitreal injections. Data<br />
were implemented in a nationwide open registry for PIOL supported by<br />
the German Federal Ministry of Education and Research.<br />
Results. Five eyes from 3 patients received a minimum of one to a