Management of NK/T-Cell Lymphoma, Nasal Type

Abstract

Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare peripheral T-cell lymphoma associated with Epstein-Barr virus. It most often presents as limited-stage disease in patients of East Asian descent with a palatal deformity caused by erosion of the tumor through the hard palate. Limited-stage disease is often curable with the use of l-asparaginase–based chemotherapy and high-dose radiation therapy. Obtaining an accurate diagnosis is essential, because treatment with standard lymphoma regimens and omission of radiation severely compromise the likelihood of long-term survival. Conversely, patients with advanced disease have a poor prognosis and are recommended for asparaginase-based chemotherapy followed by consolidation with autologous transplantation as a potentially curative approach. Progress often has been hampered by the rarity of this disease. However, discovery of common genetic alterations in pathways that promote growth and inhibit apoptosis, and actionable markers such as CD30 (among others), have begun to broaden the availability of novel drugs (eg, targeted therapies). There is also cautious optimism about immunotherapies, such as checkpoint blockade and novel cellular therapies that target Epstein-Barr virus. Advances in treatment and understanding of the genetic landscape of this disease offer hope for improved treatment outcomes.

Clinicopathological and prognostic study of primary cutaneous extranodal natural killer/T‐cell lymphoma, nasal type: A systematic review

“…ENKTL is a representative EBV‐related disease 23,97 . However, our study did not reveal any prognostic significance of HMB, which is one of the cutaneous manifestations of chronic active EBV infection, usually acquired in childhood and frequently of natural killer cell origin 2,98 .…”

“…Other baseline data included Ann Arbor stage, serum lactate dehydrogenase (LDH) level, serum EBV DNA copy number, the presence of B symptoms, International Prognostic Index (IPI) score, the presence of hemophagocytic lymphohistiocytosis (HLH), a history of hypersensitivity to mosquito bites (HMB), the immunostatus, histological features, the results of immunohistochemical studies, EBV‐encoded small RNA in situ hybridization and T‐cell receptor (TCR) gene analysis of the skin lesions, type of treatment, follow‐up duration, any progression during the follow‐up period, and survival outcome. Chemotherapies were divided into the standard chemotherapy regimens for ENKTL and others 22–24 . Specifically, standard chemotherapy regimens included asparaginase‐based chemotherapies and nonasparaginase‐based chemotherapies such as DeVIC (dexamethasone, etoposide, ifosfamide, and carboplatin), ICE (ifosfamide, carboplatin, and etoposide), DICE (dexamethasone, ifosfamide, cisplatin, and etoposide), IMEP (ifosfamide, methotrexate, etoposide, and prednisolone), ESHAP (etoposide, methylprednisolone, cytarabine, and cisplatin), and VIPD (etoposide, ifosfamide, cisplatin, and dexamethasone).…”

Natural Killer/T-Cell Lymphoma in Children and Adolescents: Clinicopathologic and Gene Expression Profiling Study of 22 Cases

“…Although some unfavourable prognostic factors, including high LDH level and non-nasal involvement at an advanced stage are reported in NKTCL of adults, and similar studies have not been analyzed well in young patients for limited samples [26,27,28,29] . In the present study, 3 out of 7 cases which refused chemotherapy or radiotherapy died of this disease.…”

Recurrent Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma with Meningeal Involvement

“…Treatment of NNKTL depends on the stage of the disease at diagnosis. It usually involves chemotherapy, radiation, and bone marrow transplantation [14]. In this case, the patient had limited-stage disease (IE).…”