Abstract
Purpose
Post-transplant lymphoproliferative disorder (PTLD) represents a spectrum of disorders associated with Epstein Barr Virus infection in up to 80% of cases in the setting of pharmacologic immunosuppression following hematopoietic stem cell or solid organ transplantation. Ocular involvement is a rare finding in PTLD.
Observation
We report the case of a 38-year-old man who presented with unilateral retinal infiltrates as first manifestation of PTLD relapse. Diagnosis relied on the presence of EBV DNA in anterior chamber fluids and vitrectomy that showed the presence of a B cell clone. Systemic relapse of PTLD was detected 12 weeks after retinal findings. Treatment of ocular disease included systemic injections of rituximab and intravitreal injections of methotrexate, halting the extension of retinal infiltrates.
Conclusion
Ocular involvement in PTLD is rare and needs to be acknowledged because it can precede a systemic relapse of the hematological condition.
1
Case report
A 38-year-old man was referred to our tertiary clinic in July 2019 for an acute visual loss of his right eye (OD). His medical history consisted in a T-cell acute lymphoblastic leukemia (ALL) for which he received allogeneic hematopoietic stem cell transplantation (HSCT) in second complete remission from an HLA-mismatch unrelated donor in March 2019. Pre-transplant serological status for Epstein-Barr virus (EBV) was positive for both the donor and recipient. At 4 months of HSCT (June 2019), he presented with fever, tonsillitis and multiple swollen lymph nodes, leading to suspect an EBV-induced post-transplant lymphoproliferative disorder (PTLD). This diagnosis was confirmed virologically by positive PCR for EBV in the blood (5,4 log UI/ml) and histologically on lymph node specimens; and treated successfully with seven rituximab injections in June and July 2019 and reduction of graft-versus-host disease (GVHD) prevention (ie. decrease of cyclosporin). At referral, the best corrected visual acuity (BCVA) was 20/40 OD and 20/20 OS (left eye). Intraocular pressure was normal and slit lamp examination of anterior segments was unremarkable. Fundus examination was normal in the left eye, but disclosed the presence of diffuse yellowish infiltrations of the posterior pole associated with a perivascular cuffing ( Fig. 1 A) in the right eye. There was no vitreous infiltration at this time. Fluorescein (FA) and indocyanine green angiography (ICG-A) showed the perivascular cuffing (dark rim) with a mild leakage at the level of both the arteries and veins, associated with a masking effect at the level of the yellow retinal infiltrates ( Fig. 2 A–D). Optical coherence tomography (OCT) showed the presence of a subretinal infiltration, and a serous retinal detachment of the fovea ( Fig. 2 E). An anterior chamber tap (ACT) was performed and came back negative for PCR and Goldmann-Witmer coefficient of HSV 1 and 2, VZV, CMV, EBV and toxoplasmosis; and dosage of interleukin 10 (IL-10). Four weeks later, BCVA of his right eye dropped to counting fingers corresponding to an enlargement of the yellowish dot shaped retinal infiltrations ( Fig. 1 B–C) appearing this time as drusen like deposits on OCT ( Fig. 2 F). At this point, the two main hypotheses were an ocular PTLD or an ocular relapse of his ALL (since there was no systemic sign of disease). Iterative ACTs were persistently negative for infectious diseases and IL-10 except in August 2019 when a PCR performed on the aqueous humor came back positive for EBV DNA (4.77 Log UI/mL). Considering the poor visual outcome, and despite the absence of vitritis or vitreous cells, a diagnostic vitrectomy was performed in November 2019, showing the presence of a B cell clone suggestive of an ocular PTLD. The B cell clone presence in the vitreous was revealed by PCR amplification. At the same time, EBV DNA was detected in several blood samples and in a liver biopsy confirming a subsequent relapse of the systemic disease while the patient was treated for gut and skin steroid-refractory acute GVHD. Systemic treatments including rituximab, brentuximab-vedotin and chemotherapy were administered with partial control of the disease initially, followed by progression despite successive lines of treatment until the death of the patient in July 2019. Locally he was treated with three intravitreal injections of methotrexate halting the extension of PTLD infiltrates without relapsing until the death of the patient ( Fig. 1 E). Despite the good anatomic response to treatments, no visual improvement in his best corrected visual acuity was noted overtime.
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