2018, Number 3
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ABSTRACTHydroa vacciniforme–like cutaneous T-cell lymphoma (HVLCTCL) is a clinical entity characterized by malignant proliferation of T lymphocytes with or without angiocentric invasion of blood vessels; it has an unusual presentation. Its frequency is greater in pediatric population of Mexico, Central and South America, and parts of Asia. It appears mainly in areas exposed to light, such as face, back of hands, arms and legs, with exudative and crusted papular vesicles on an erythematous base that leave varioliform scars similar to hydroa vacciniforme. In addition, facial edema can occur, as well as infiltrative nodules and suppurative ulcerative lesions. Infections with Epstein-Barr virus (EBV) have been commonly associated with T or NK cell monoclonal lymphoma with a high malignancy rate, resistance to chemotherapeutic agents and high mortality. We describe the case of a seven-year-old patient who suffered from HVLCTCL; his diagnosis was very difficult because of the complexity of the histopathological study. The patient reported belongs to a rural area in Quito, Ecuador.
Sawada A, Inoue M, Koyama-Sato M, Kondo O, Yamada K, Shimizu M et al. Umbilical cord blood as an alternative source of reduced-intensity hematopoietic stem cell transplantation for chronic Epstein-Barr virus-associated T or natural killer cell lymphoproliferative diseases. Biol Blood Marrow Transplant. 2014; 20 (2): 214-221.