Extranasal natural killer (NK)/T-cell lymphoma and aggressive NK-cell leukemia are strongly associated with Epstein-Barr virus (EBV) and most often occur in middle-aged individuals. Overlap between these 2 diagnoses is rare. In addition, pathologic findings for these 2 diagnoses are typically notable for necrosis, apoptosis, angioinvasion, and angiodestruction.
We describe a 15-year-old male adolescent who had painful subcutaneous nodules and plaques over his anterior thighs, shins, and lower abdomen while receiving anti-tumor necrosis factor therapy with infliximab. He also was noted to have pulmonary nodules, liver nodules, hepatosplenomegaly, thrombocytopenia, and transaminitis. A skin biopsy revealed atypical small to intermediate-sized EBV-positive lymphoid cells of NK-cell origin infiltrating the subcutaneous adipose tissue, mimicking subcutaneous T-cell lymphoma. Similar atypical EBV-positive lymphocytes were noted in the bone marrow, liver, stomach, and colon. This patient had a rapidly fatal disease course.
We report a unique clinical and histological presentation most consistent with an extranasal NK/T-cell lymphoma and aggressive NK-cell leukemia overlap, although our case may represent a disease entity completely new to the literature. In addition, we report the first case to our knowledge of an EBV-positive NK/T-cell lymphoma developing in the setting of tumor necrosis factor inhibitor therapy.
[Show abstract][Hide abstract] ABSTRACT: Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL). A case of cutaneous ENKLN and a case of ANKL in Thai patients are reported Both patients developed hemophagocytic syndrome and shortly succumbed to death. The cells in cutaneous ENKLN are small to medium in size with minimal cytoplasm, round nuclei, irregular nuclear membrane, andfine chromatin with inconspicuous nucleoli. While that of ANKL are medium to large-sized mononuclear cells with moderate cytoplasm. Their nuclei are elongated to embryo-like with irregularly thickened nuclear membrane, fine chromatin, and small to occasional prominent nucleolus. Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization. The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.
Journal of the Medical Association of Thailand = Chotmaihet thangphaet 06/2007; 90(5):982-7.
[Show abstract][Hide abstract] 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.
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