Multimodal Imaging Features of Retinal Lesions in Hemophagocytic Lymphohistiocytosis: A Clinical Analysis.

OBJECTIVE

Hemophagocytic lymphohistiocytosis (HLH) associated retinopathy remains poorly characterized in terms of clinical phenotypes and pathogenesis. This retrospective study aimed to systematically define the multimodal imaging features of retinal lesions in patients with HLH and investigate its underlying pathological mechanisms to inform clinical practice.

METHODS

A retrospective case series analysis was conducted on 15 HLH patients with retinal lesions. Comprehensive ophthalmic evaluations, including fundoscopy and optical coherence tomography (OCT), were integrated with systemic multimodal imaging data.

RESULTS

Imaging analysis revealed characteristic retinal structural alterations, including retinal hemorrhage (26 eyes, 86.67%), outer retinal atrophy (20 eyes, 66.67%), outer retinal hyperreflective foci (7 eyes, 23.33%), ellipsoid zone disruption (17 eyes, 56.67%) and ellipsoid zone thinning (10 eyes, 33.33%). Multimodal imaging findings suggest a potential association between HLH related retinopathy and a hypoperfused ischemic state of the retina, though this requires further validation through larger scale statistical analysis. Notably, characteristic damage to the outer retinal structures was observed across HLH patients of different etiological subtypes and could manifest at any stage of the disease, including before and after interventions such as chemotherapy and hematopoietic stem cell transplantation.

DISCUSSION: The study data indicate potential involvement of HLH induced systemic cytokine storms, secondary hemoglobin reduction, and hematologic abnormalities such as thrombocytopenia in the observed retinal changes. These interpretations should be understood as hypothesis generating observations within the constraints of descriptive research methodology. Therefore, while cytokine cascade control remains a cornerstone of management, future studies should explore the potential benefit of blood component supplementation as an adjunctive therapy in HLH related retinopathy.

CONCLUSION

These findings highlight the necessity of establishing early warning indicators for HLH associated retinopathy and conducting multicenter prospective studies to optimize evidence based diagnostic and therapeutic strategies.