Indolent CD30-Positive Extranodal NK/T Cell Lymphoma with Large Cell Transformation: Case Report and Literature Review
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CLINICAL REPORT
Indolent CD30-Positive Extranodal NK/T Cell Lymphoma with Large Cell Transformation: Case Report and Literature Review Pengyi Yu1 • Tao Tang2 • Yan Tan3 • Hui Wang3 • Qing Li3
Received: 26 June 2020 / Accepted: 28 September 2020 Ó Association of Otolaryngologists of India 2020
Abstract The nasal type of extranodal natural killer (NK)/ T-cell lymphoma (ENKL) is an aggressive form of nonHodgkin’s lymphoma. Although ENKL is most commonly seen in the midline of the nose, face and Waldeyer’s ring, it can also occur in the skin, gastrointestinal tract, soft tissues and other parts of the body. Severe ENKL cases are accompanied by hemophagocytosis, with clinical manifestations such as high fever, hepatosplenomegaly, and decreased blood cell count. ENKL at different locations exhibits similar histological features and immunophenotypes, such as a strong affinity for T cell markers CD2 and CD56, cytotoxic molecules, as well as a strong positive for EBER after in situ hybridization. Although indolent ENKL is extremely rare, we hereby present a case study of primary NK/T cell lymphoma in the spinal canal with the initial manifestation of a diffuse growth of small cells, and the survival and recurrence details after 11 years, accompanied by CD30-positive large cell transformation. The patient’s condition after treatment has improved and is currently in good health. Pengyi Yu and Tao Tang have contributed equally to this work. & Hui Wang [email protected] Qing Li [email protected] 1
Department of Thoracic Surgery, The Third Affiliated Hospital of Soochow University, 185 Juqian Street, Changzhou 213003, People’s Republic of China
2
College of Life Sciences, Nanjing Normal University, 1 Wenyuan road, Nanjing 21004, People’s Republic of China
3
Department of Pathology, The Third Affiliated Hospital of Soochow University, 185 Juqian Street, Changzhou 213003, People’s Republic of China
Keywords Extranodal NK/T-cell lymphoma Nasal type Large cell transformation CD30-positive Epstein-barr virus
Introduction Extranodal natural killer (NK)/T-cell lymphoma (ENKL) is an aggressive form of non-Hodgkin’s lymphoma that is associated with Epstein-Barr virus infection. Although ENKL is most commonly seen in the nose, facial midline, and the Waldeyer’s ring, it can also occur in the skin, gastrointestinal tract, soft tissue, brain, liver, spleen, bone marrow, and testis [1]. Severe ENKL often manifests in conjunction with hemophagocytic syndrome with clinical manifestations of high fever, hepatosplenomegaly, and hemophagocytosis. ENKL sites, though diverse, possess similar histological features such as extensive necrosis and inflammatory exudates as well as immunophenotypes. Tumor cells are either round or ovoid and medium to large in size, with a moderate amount of cytoplasm, an eggplant or kidney-like irregular nucleus, and an inconspicuous nucleolus [2]. They often intermix with anaplastic cells, such as small to medium atypical lymphoid cells, histiocytes, and eosinophils, and proliferate around blood vessels,
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