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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

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