Primary Cutaneous Extranodal Natural Killer (NK)/T-cell Lymphoma, nasal type (PC-ENKTL), is a rare entity of malignancies of NK cells or cytotoxic T cells, characterized by an association with Epstein-Barr virus (EBV) infection. Despite its aggressive behavior, PC-ENKTL is mostly found as a localized disease. Data regarding PC-ENKTL with generalized skin lesions have only rarely been characterized in clinical studies so far. We present a case of PC-ENKTL, nasal type, in a 38-year–old female with a history of disseminated cutaneous plaques, ulcers and painful nodules that originated in her right leg skin as a small raised papule and expanded quickly over the entire body, ranging in diameter from 1 cm to 6 cm. The patient did not show any involvement of other sites except skin. Histologic, immunophenotypic, genetic and clinical features consistent with the diagnosis of primary cutaneous NK/T-cell lymphoma, nasal type. The status of immune cells was analyzed using a panel of monoclonal antibodies and revealed negative stain for CD163, CD68, programmed cell death (PD-1)/Programmed cell Death l Ligand 1(PD-L1), and FoxP3. Chemotherapy followed by radiotherapy was planned but the patient was experienced disease progression and died at 3 months as a result of lymphoma. In contrast to NK/T-cell lymphoma with localized disease, PC-ENKTL with generalized skin lesions tends to be more aggressive, with short survival and extremely poor response to therapy. We propose that the immune cell shift may be related to the severity of the patient.
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Journal of Blood & Lymph received 443 citations as per Google Scholar report
