Bilateral exudative retinal detachment as a presenting sign of acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) can present with various ocular complications but exudative retinal detachment is a rare complication. A 36-year-old healthy young adult male presented with gradual decrease in the vision in both eyes over nearly 2 weeks. His best-corrected visual acuities were 20/50 and 20/25 at distance and N12 and N10 at near in the right and left eyes, respectively. Fluorescein angiography and optical coherence topography indicated bilateral exudative retinal detachment. Systemic workup revealed a marked increase in the number of white blood cells with 30% blast cells and immunophenotyping revealed common acute lymphoblastic leukemia-associated antigen (CALLA) positive precursor B-cell lymphoblastic leukemia. Cerebrospinal fluid (CSF) tap was negative. The patient started systemic chemotherapy and steroids. Bilateral exudative retinal detachment may be a presenting sign of acute lymphoblastic leukemiaALL in an otherwise healthy young adult. Clinicians should be aware of the possibility of leukemia in such patients. A simple blood investigation such as complete blood profile confirms the diagnosis.

INTRODUCTION

Exudative retinal detachment, a very rare ocular finding in leukemia, has only been described in a few cases and has not been previously reported in India.12 This case is also the first case to document common acute lymphoblastic leukemia-associated antigen (CALLA) positive precursor B-cell lymphoblastic leukemia manifesting as exudative retinal detachment in an otherwise healthy young adult male from south India.

CASE REPORT

A previously healthy 36-year-old male presented with gradual decrease in near vision in both eyes over 2 weeks. There was no history of any systemic disease, steroid intake, ocular trauma, or surgery. His best corrected visual acuities (BCVAs) were 20/50 and 20/25 at distance and N12 and N10 at near vision in the right and left eyes, respectively. There was no anterior chamber reaction or vitreous cells in either eye. Fundus examination showed multifocal bilateral exudative retinal detachment in both eyes [Figure 1]. Fluorescence angiography (FA) in the early phase showed delayed choroidal filling and pinpoint hyperfluorescence at the level of the retinal pigment epithelium [Figure 2a]. Late phase FA was apparent for pinpoint hyperfluorescent dots remaining the same in size and intensity [Figure 2b]. Optical coherence tomography (OCT) revealed multiple areas of subretinal serous fluid pockets in both eyes [Figure 3]. A clinical diagnosis of bilateral exudative retinal detachment was considered. However, systemic evaluation revealed increased white blood cell counts (2,90,000/μL), with an abnormally increased percentage of blast cells (30%). Immunophenotyping confirmed the diagnosis of CALLA positive precursor B cell lymphoblastic leukemia. However, cerebrospinal fluid (CSF) tap was negative. The patient was started on systemic chemotherapy of vincristine, prednisolone, daunorubicin, l-asparaginase (VPDL) and high-dose steroid therapy, prednisolone 60 mg daily by the oncologist. The patient was lost to follow-up.

Figure 1

Fundus examination with multifocal exudative retinal detachment in both eyes

Figure 2

(a) Fluorescence angiography (FA) in the early phase showed delayed choroidal filling and pinpoint hyperfluorescence at the level of the retinal pigment epithelium in right eye, (b) Fluorescence angiography (FA) in the early phase showed delayed choroidal filling and pinpoint hyperfluorescence at the level of the retinal pigment epithelium in left eye, (c) Late phase FA was apparent for pinpoint hyperfluorescent dots remaining the same in size and intensity in right eye, (d) Late phase FA was apparent for pinpoint hyperfluorescent dots remaining the same in size and intensity in left eye

Figure 3

(a) Optical coherence tomography (OCT) revealed multiple areas of subretinal serous fluids in left eye, (b) Optical coherence tomography (OCT) revealed multiple areas of subretinal serous fluids in right eye

DISCUSSION

In leukemia patients, vision loss due to choriocapillary occlusion was first reported by Zimmerman in 1964.1 Beyond that, exudative retinal detachment in leukemia has been reported in only a few cases worldwide, as a presenting sign of the disease or the first sign of relapse.1–5 Our case presented with bilateral exudative retinal detachment, with multiple pinpoint hyper- fluorescent areas on fundus fluorescein angiography fundus fluorescein angiography (FFA) with features resembling Vogt Koyanagi Harada (VKH) disease. We ruled out VKH due to the absence of disc leakage and absence of inflammatory signs. This VKH-like exudative retinal detachment as a presenting sign of leukemia has not been reported from India.

Bilateral exudative retinal detachment in a young male can be due to central serous chorioretinopathy (CSC), however, our patient did not show any features of CSC leaks on FFA. He did not report any risk factor for CSC such as history of steroid usage. Other causes for exudative retinal de tachment such as renal or cardiac dysfunction have been ruled out by systemic investigations. The complete blood picture alone helped to diagnose the underlying leukemia in our patient.

Causative factors leading to exudative retinal detachment in leukemia has been thought to be choroidal ischemia and secondary retinal pigment epithelial dysfunction.3467 The choroid is the most frequently involved ocular tissue in leukemia. Leukemic cell infiltration or hematologic disturbances may cause partial occlusion of the choriocapillaries and delay of choroidal circulation.8 Secondary dysfunction of the Bruch's membrane and retinal pigment epithelium may ultimately lead to exudative retinal detachment of the macula.910 Histologically, it is confirmed by autopsy that in leukemic patients choroidal thickening and leukemic cell infiltration are the factors causing choroidal ischemia.8 Leukemia may present when there is sudden visual loss due to exudative retinal detachment, and the ophthalmologist may be the first clinician to encounter such patients. A thorough systemic evaluation should be performed in patients presenting with bilateral exudative retinal detachment, especially in those who are otherwise healthy.