Abstract
Recently established by the World Health Organization classification, "nasal" and "nasal-type" NK/T-cell lymphoma arise from natural killer (NK) cells. They have distinct clinicopathologic features, specific genotype and phenotype, and a high association with Epstein-Barr virus infection. Nasal-type NK/T-cell lymphoma arise from extranasal sites including skin, soft tissue, gastrointestinal tract, liver, spleen, testes, lung, and central nervous system. Most cases are reported from Asia and South America in adults. There are very few cases reported in the pediatric age group. We report an 11-year-old child with extranodal nasal-type NK/T-cell lymphoma presenting as hemophagocytic syndrome and multiorgan system failure with fatal outcome. © 2008 Lippincott Williams & Wilkins.