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4 th October 2007 Jamie Ng. Grand Ward Round. The Eye Institute Tan Tock Seng Hospital. History. Mr CYO 37/Chinese/Male Medical history Obstructive sleep apnoea Obese Chronic sputum – 2yrs with occ haemoptysis Prev investigated and treated with course of anti-biotics, improved
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4th October 2007 Jamie Ng Grand Ward Round The Eye Institute Tan Tock Seng Hospital
History • Mr CYO 37/Chinese/Male • Medical history • Obstructive sleep apnoea • Obese • Chronic sputum – 2yrs with occ haemoptysis • Prev investigated and treated with course of anti-biotics, improved • Currently on TCM • Multiple skin lumps for many years (Father also has similar lumps)
Presenting Complaints • Left eye BOV for 2/12 • Prominence of left eyeball for 9/12 • Gradually increasing • Also noticed that his short-sightedness became less since 4/12 ago • No pain • Systemic review essentially normal
Examination • VR - 6/6 VL – 6/24 → 6/12
108 18 22 Examination • Fine tremors and warm palms noted • Left axial proptosis • Mild chemosis LE • GAT 14 mmHg OU
Examination -1 0 -1 0 0 0 0 0 • EOM
Examination • No RAPD • Ishihara R 15/15 L 15/15 • Light brightness – R 100% L 80% • No red desaturation • Confrontation - BE no gross field defects • Anterior Segment BE otherwise normal
Examination • Posterior segment
Differential diagnosis • Thyroid eye disease • Tumours – intraconal • Optic nerve meningioma • Optic nerve glioma • Orbital lymphoma • Cavernous hemangioma • Orbital varix • Carotid Cavernous Fistula • Metastatic disease
Investigations • Blood investigations • TSH, T3, T4 and TSH receptor Antibody – normal • FBC, U/E/CR, ESR and syphillis screen - normal • Imaging • CT orbits 18/09/07
Investigations • MRI Orbits 20/09/07
Subsequently…… • Left grade 1 to 2 RAPD • Ishihara R 15/15 L 13/15 • Light brightness – R 100% L 70% • Left intraconal orbital mass with early compression of optic nerve • Referred to OPLS for biopsy of lesion via lateral orbitotomy
Subsequently…… Review of films and patient:
Ocular Lymphoma • 1. Lymphoma of ocular adnexa • 2. Primary intra-ocular CNS lymphoma • Diffuse large B-cell NHL • Rare • A. Solitary or multiple intracranial nodules • B. Diffuse meningeal lesions • C. Localized intradural masses • D. Intraocular involvement • Poor prognosis
Ocular Adnexal Lymphoma Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Jenkins C, Rose GE, Bunce C, Wright JE, Cree IA, Plowman N, Lightman S, Moseley I, Norton A. Orbital Clinic, Moorfields Eye Hospital, London. Br J Ophthalmol. 2000 Aug;84(8):907-13
Ocular Adnexal Lymphoma • The biopsies and clinical follow up data for 192 patients with ocular adnexal lymphoma were reviewed • Classified according to the Revised European American Lymphoma (REAL) system • 1. Marginal Zone Lymphoma • 2. Lymphoplasmocytic lymphoma • 3. Follicle center lymphoma • 4. Diffuse large B cell lymphoma • 5. Plastocytoma and other rare histologic variants
Ocular Adnexal Lymphoma Marginal Zone Lymphoma Follicle Center Lymphoma Diffuse Large B-cell Lymphoma Atypical Mantle Cell
Ocular Adnexal Lymphoma • Treatment : All but 4 patients received local DXT and/or various combinations of chemotherapy • Mean time to relapse in orbital MZL – 63 months • MZL - considered indolent and have a good prognosis • Provides evidence showing good correlation of histoloical grade and survival outcome in ocular adnexal lymphoma
Ocular Adnexal Lymphoma • Management • Correct histological diagnosis and accurate staging • Localized low grade – • up to 90% controlled with DXT • DXT is often well tolerated • Intermediate to high grade – combined with adjuvant chemotherapy • Localized orbital lymphoma. Galieni P, Polito E, Leccisotti A, Marotta G, Lasi S, Bigazzi C, Bucalossi A, Frezza G, Lauria F. Haematologica. 1997 Jul-Aug;82(4):436-9. • Radiotherapy for Orbital Lymphoma : Outcome and Late Effects. Bischof M, Karagiozidis M, Krempien R, Treiber M, Neuhof D, Debus J, Zierhut D. Strahlenther Onkol. 2007 Jan;183(1):17-22
Interesting reports • Concomitant HCV infection is associated with more disseminated disease and aggressive behavior in OAL. • HCV seropositivity is present in 13% of OAL of MALT-type. • Clinical implications of hepatitis C virus infection in MALT-type lymphoma of the ocular adnexa.Ferreri AJ, Viale E, Guidoboni M, Resti AG, De Conciliis C, Politi L, Lettini AA, Sacchetti F, Dolcetti R, Doglioni C, Ponzoni M. Ann Oncol. 2006 May;17(5):769-72. Epub 2006 Mar 8 • Chlamydia psittaci infection may contribute to the development of lymphomas, as was also supported by the clinical responses observed in this study with C. psittaci-eradicating antibiotic therapy. • Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. Ferreri AJ, Guidoboni M, Ponzoni M, De Conciliis C, Dell'Oro S, Fleischhauer K, Caggiari L, Lettini AA, Dal Cin E, Ieri R, Freschi M, Villa E, Boiocchi M, Dolcetti R. J Natl Cancer Inst. 2004 Apr 21;96(8):586-94
Thank You The Eye Institute - Jamie Ng