Nasal-type extranodal NK/T cell lymphoma in association with hemophagocytic syndrome: a case report and literature review*

Abstract

Abstract We present a rare case of nasal-type CD56-negative NK/T-cell lymphoma. The patient developed hemophagocytic syndrome during diagnosis and treatment. The patient presented to our hospital (Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China) with “nasal congestion for 3 months and scattered erythema, nodules, and ulcers all over the body for 1 month.” We analyzed clinical manifestations, skin histopathology, immunohistochemistry, and in situ hybridization results. Histopathology of the skin revealed a moderate amount of atypical lymphocyte infiltration between the entire dermis and collagen bundles. Immunohistochemistry showed the following: CD30 (+), TIA-1 (+), CD3(2GV6) (+), CD5 part (+), CD8 part (+), CD43 (+), CD56 (-), CD4 (-), CD20 (-), PAX5 (-), PCK (-), P63 (-), P40 (-), EGFR (-), Ki-67 (the hot spot LI is approximately 80%), and in situ hybridization EBER-ROCH (+). The diagnosis made was “NK/T cell lymphoma nasal type”. This type of lymphoma is aggressive, progresses quickly, and has a poor prognosis. Early clinical manifestations are extremely atypical, especially in the absence of rash. Analysis of the skin manifestations of the disease has a positive effect on its early diagnosis, early treatment, and prognosis.