To present a rare case of bilateral peripapillary retinal leukemic infiltration associated with papilledema without retrobulbar optic nerve involvement in a patient with acute lymphoblastic leukemia (ALL).
This was a retrospective case report.
A 24-year-old man with T-cell ALL and 2-month history of papilledema presented to our department with reduction of visual acuity in the left eye. Visual acuity was 20/60 in the right eye and hand movements in the left eye. Fundus examination revealed grade 3 papilledema, bilateral peripapillary leukemic infiltration, and intraretinal hemorrhages. Cranial magnetic resonance imaging did not show any sign of cranial mass or optic nerve involvement. Pulse steroid therapy and oral acetazolamide was started. At day 18, visual acuity improved to 20/40 in the right eye and 20/60 in the left eye, while papilledema improved to grade 1 and hemorrhages regressed.
To our knowledge, there is no other report of peripapillary leukemic infiltration in the absence of retrobulbar optic nerve involvement. We suspect that papilledema might have facilitated peripapillary retinal infiltration due to altered vascular permeability. The reverse could also be possible: leukemic infiltration leading to increase in vascular permeability may also contribute to papilledema.
Tumori 2016; 102(Suppl. 2): e128 - e130
Article Type: CASE REPORT
AuthorsOsman Çekiç, Esra Biberoglu, Fehim Esen
- • Accepted on 09/02/2016
- • Available online on 14/03/2016
- • Published online on 11/11/2016
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Ocular involvement in patients with acute leukemias is not rare. Ocular involvement was found to be present in 17% of childhood acute leukemia cases, while only 3.6% of the children were symptomatic (1). In that series, the most commonly observed ocular finding was intraretinal hemorrhages (11%) and papilledema was only observed in a single case among 82 children with leukemias (1.2%) (1). In acute leukemia cases, papilledema has been reported to be mostly associated with cranial involvement (2, 3), optic nerve involvement (4, 5), optic perineuritis (6), or medications causing pseudotumor cerebri (7). Here, we report a rare case of bilateral peripapillary retinal leukemic infiltration associated with preexisting papilledema, in the absence of the above-mentioned predisposing factors, in a patient with acute lymphoblastic leukemia (ALL).
A 24-year-old man was referred to our clinic for routine ophthalmologic examination before initiation of the fourth course of hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) chemotherapy. The patient did not have visual complaints before and had been diagnosed 3 months previously with T-cell ALL. The initial bone marrow biopsy revealed blastic cell infiltration with small nuclei, thick chromatin, and vacuolated cytoplasm, which constituted 70% of the cells in the bone marrow. Molecular cytogenetic screening was performed for t(15;17)(q22;q21.1), inv(16)(p13q22), t(8;21)(q22;q22), t(9;22)(q34;q11.2) with fluorescein in situ hybridization; the results were negative. The blastic cells expressed CD3, TdT, and CD34 and did not express CD20 or PAX5. On complete blood count, the hemoglobin was 11.0 g/dL, white blood cell count (WBC) was 290,000/mm3, and platelets were 13,000/mm3 at the time of diagnosis. His WBC count was 6,300/mm3 during his first ophthalmologic examination under hyper-CVAD regimen. Best-corrected Snellen visual acuity was 20/20 bilaterally. Color vision score with Ishihara charts was 11/20 in the right and 9/20 in the left eye. Intraocular pressure was 15 mm Hg in the right eye and 15 mm Hg in the left eye. Bilateral anterior segment examination was nonspecific. Light reflexes were bilaterally normal and no relative afferent papillary defect was observed. Bilateral fundus examination revealed grade 4 papilledema and some flame-shaped hemorrhages along the superotemporal arcades (
At the second month of follow-up, the patient presented with reduced vision: visual acuity was 20/60 in the right eye and hand movements in the left eye. Massive bilateral peripapillary retinal leukemic infiltration was observed with indirect ophthalmoscopy (
Papilledema is a rare finding associated with ALL. In the literature, there are few cases reporting direct infiltration of the optic nerve by the leukemic cells (4, 5) or optic perineuritis (6), describing either cases with associated pseudotumor cerebri (2) or papilledema due to a mass lesion in the brain (3). In our case, peripapillary leukemic infiltration was not associated with the involvement of the rest of the optic nerve or any cerebral mass lesion. In the previous reports of papillary infiltration together with optic nerve or cranial involvement, early initiation of systemic chemotherapy, intrathecal methotrexate, or cranial radiotherapy was suggested along with high-dose intravenous methylprednisolone and intrathecal hydrocortisone treatment to reduce optic nerve swelling (5). Our case did not have such a clinical picture and was already treated systemically. We decided to start pulse steroid treatment in order to reduce vascular permeability and subsequent tissue edema endangering the viability of retinal nerve fibers. This ocular condition responded well to systemic steroid treatment.
We observed peripapillary infiltration of the leukemic cells, under the clinical picture of preexisting papilledema. It seems that papilledema facilitated retinal infiltration by altering vascular permeability. The reverse could also be possible: leukemic infiltration-related increase in vascular permeability might have resulted in papilledema. Because papilledema preceded peripapillary retinal infiltration, and no central nervous system or retrobulbar involvement was detected by cranial magnetic resonance imaging, the first hypothesis seems more consistent with the presented case.