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Revista chilena de pediatría

versão impressa ISSN 0370-4106

Resumo

SILVA FEISTNER, Marcos; ROJAS LECHUGA, María Jesús; ORTIZ, Elena  e  MOLGO, Montserrat. Extranodal Natural Killer/T-Cell Lymphoma, nasal-type with primary cutaneous involvement. A case report. Rev. chil. pediatr. [online]. 2018, vol.89, n.2, pp.246-250.  Epub 04-Abr-2018. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062018000200246.

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 or obstruction of the airway. Also, may be primary skin involvement, airway and other organs.

Objecti ve:

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 pedia tric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression.

Conclu sions:

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.

Palavras-chave : Extranodal natural killer/T-cell lymphoma nasal type; pediatric; Ebstein-Barr virus.

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