Corneal perforation in the setting of NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis.

BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe systemic inflammation caused by pathologic immune dysregulation and resulting in multiorgan destruction4. While ocular manifestations are recognized in HLH, corneal perforation has not been well-described in published reports.

CASE PRESENTATION: We report a case of a 26-year-old male who developed a spontaneous corneal perforation in the setting of NK/T-cell lymphoma-associated HLH. The patient was transferred to our hospital with a progressively enlarging fungating facial mass and severe bilateral eyelid edema, as well as fevers, sepsis, pancytopenia and transaminitis. Biopsy of the facial mass was consistent with extranodal natural killer/T-cell lymphoma (ENKTL), and the patient was diagnosed with HLH presumed secondary to his malignancy. Ophthalmic examination revealed a corneal perforation of his left eye.

MANAGEMENT AND OUTCOME: The patient was taken to the operating room for repair of the perforation with a corneal patch graft. Postoperative management included topical corticosteroids and antibiotics. After surgery, the patient's left eye visual acuity was 20/100. Three months later, his visual acuity was 20/30 and the corneal patch graft appeared well-healed.

CONCLUSION

This case highlights the potential for HLH to cause corneal perforation as a severe ocular complication. Prompt diagnosis and multidisciplinary management are crucial for optimizing outcomes in such complex cases.