Extranodal Natural Killer/T-Cell Lymphoma, nasal-type with primary cutaneous involvement. A case report

Abstract

Introduction: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum orobstruc tion of the airway. Also, may be primary skin involvement, airway and other organs.

Objective: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists.

Case Report: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatriconcology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression.

Conclusions: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.