In a post-treatment non-Hodgkin's lymphoma (NHL), ocular lymphoma may be the first sign of a systemic relapse.1 We report, probably the first case of a benign systemic NHL-in-remission, causing central retinal artery and vein occlusion, without any other associated central nervous system (CNS) involvement.
A 28-year-old man presented with abdominal pain to his physician. There was no history of weight loss or fever. Ultrasonography of abdomen showed hepatomegaly and abdominal lymphadenopathy. Systemic examination showed palpable axillary lymph nodes. Fine-needle aspiration cytology from the left axillary lymph nodes showed a nodal marginal zone B-cell NHL. Bone marrow biopsy was normal. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone were given. The patient responded well, with complete remission of the lymphoma, radiologically and ultrasonographically.
Four months later, his right eye suffered a sudden drop in visual acuity to light perception; the left eye was 20/20. An afferent pupillary defect was noted in the right eye. The anterior segment and angles were unremarkable on slit-lamp biomicroscopy and gonioscopy in either eye. Intra-ocular pressure was normal in both the eyes. Fundus examination of the right eye showed a grossly swollen optic disc, pale macular edema with a cherry-red spot, venous tortuosity and extensive retinal hemorrhages [Figure 1a]. Fluorescein angiography showed no retinal vascular perfusion [Figure 1b]. Left fundus was normal. Magnetic resonance imaging (MRI) and computerized tomography (CT) showed thickening of the right optic nerve and enhancement of the optic-nerve-sheath complex in a post-contrast study [Figure 2]. CNS was not involved. Chest X-ray, abdominal ultrasound and CT failed to detect any systemic recurrence. Intravenous methyl prednisolone (15 mg/kg/day) was given for three days, followed by oral prednisolone, 1 mg/kg. The patient was referred to an oncologist for radiotherapy of the optic nerve. Two months later, he returned with pain in right eye. The vision remained light perception. Anterior segment examination showed a steamy cornea, a persistent afferent pupillary defect and rubeosis of the iris and angles. Intraocular pressure was 40 mmHg by applanation tonometry. Fundus showed reduced severity of hemorrhages and disc edema. Panretinal photocoagulation was done to treat the neovascular glaucoma. The rubeosis and pain regressed; a stable ocular and systemic status was observed for the next 12 months.
NHLs are a heterogeneous group of malignancies of the lymphoid system. The WHO classification of hematopoietic and lymphoid tumors classifies lymphomas into B-cell and T-cell neoplasms; the former account for more than 85% of all lymphomas.2 Intraocular lymphomas are usually B-cell neoplasms.1 While retinal hemorrhages and cotton-wool spots may occur due to anemia or thrombocytopenia, direct retinal involvement is extremely rare in systemic NHL.3
There are three reports of combined vascular occlusion, secondary to NHL, in the literature.456 These occlusions were primarily due to lymphomatous infiltration of the optic nerve. Septic emboli and paraneoplastic hypercoagulability were the additional causes. We also suspected an infiltrative optic neuropathy, with subsequent infiltration/compression of the vascular wall, in our case. Similar to the report of Saatci et al.,4 our case also experienced a vascular occlusion during disease remission. However, there were some distinctive features: In spite of a well-differentiated systemic B-cell lymphoma, our patient suffered a severe combined vascular occlusion, with neovascular glaucoma. We were unable to find such an association in the literature; the above-mentioned combined occlusions occurred due to large/mixed B-cell or T-cell lymphoma. Further, in the previous reports of optic nerve infiltration and combined vascular occlusion in the background of regressing NHL, CNS was involved in the relapse.45 A systemic/CNS relapse of lymphoma did not occur in our patient. Optic neuropathy may occur as the sole manifestation of NHL recurrence.7
We observed combined vascular occlusion as the only manifestation of recurrent systemic NHL. Visual prognosis was poor in spite of aggressive treatment with corticosteroids and irradiation. Even a benign systemic NHL can be deceptively destructive to the eyes; it is important to explain the guarded visual prognosis to the patient, even after achieving successful remission of the tumor.
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