Purtscher Retinopathy as a Manifestation of Hemophagocytic Lymphohistiocytosis

Abstract

The authors describe a patient with hemophagocytic lymphohistiocytosis, a disease characterized by disruption of normal natural killer cell activity with subsequent uncontrolled cytokine release, who presented with Purtscher retinopathy confirmed with spectral-domain optical coherence tomography.

Ophthalmic artery ischemia in hemophagocytic lymphohistiocytosis: A case report and review of current literature

“…Posterior segment findings are well documented in the literature. Retinal hemorrhages and VH are some of the most common manifestations of HLH, and these are in keeping with the fundus examination in our case [4][5][6][7][8][9][10][11][12][13][14] (Figures 1A, B and 3A, B). However, our FFA images reveal reduced retinal filling and poor choroidal filling (Figure 2A and B), not previously identified in the literature.…”

“…Literature summary of published case reports[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] …”

Primary Intraocular Lymphoma in a Patient with Bilateral Epstein-Barr Virus Panuveitis: A Case Report

“…The authors cited a previous report presented by Lee and Mieler on uveitis and exudative detachment in a patient with HLH 14 . Others have described rare ocular involvement in HLH to include panuveitis 15 and Purtscher-like retinopathy 16 .…”

Ischemic Retinal Vascular Disease in an Infant With Hemophagocytic Lymphohistiocytosis

“…To our knowledge, there are only 14 published case reports of ophthalmic findings in patients with HLH manifesting with retinal hemorrhages, retinal detachment, uveitis, pigment epithelial detachment, macular and optic disc edema, and Purtscher-like retinopathy . Only 4 of these 10 cases were described during infancy, and 3 of these infants presented with papilledema (1 of whom also had vitreous haze and opacities) and the other presented with bilateral white spots deep in the retina.…”