NK/T-cell Lymphoma: Report Of 2 Cases And Literature Review

Objective To investigate the clinical features, diagnosis and treatments of NK/T cell lymphoma.Methods The clinical manifestations, diagnoses, and therapies of two patients with NK/T cell lymphoma were analyzed and then the literature were reviewed.Result Two patients with NK/T cell lymphoma were admitted to our hospital between January 1,2000 and December 31,2009. One is nasal NK/T cell lymphoma, the other is nase-type NK/T cell lymphoma. Nasal neoplasma, soakage of abnormal lymphoid cells and broad coagulation necroses were found in the first patient, and the abnormal lymphoid cells expressed CD56, CD45RO, cytoplasmic CD3, TIA-1, granzyme B, and EBER was positive. Skin lumps and ulcers, infiltration of profiled lymphoid cells with angiocentricity and angiodestruction were detected in the second patient. The abnormal lymphoid cells expressed cytoplasmic CD45RO, CD3, TIA-1, granzyme B, without CD56, and EBER was positive. The efficacies of chemotherapies were better in the first patient. By the end of follow-up period, he has survived more than seven years. Although a series of chemotherapies and autologous stem cell transplantation were used, the therapeutic efficacies were poor in the second patient and she eventually died of infectious shock and septicemia. She had survived thirteen months.Conclusion NK/T cell lymphoma is a rare entity of non-Hodgin lymphoma. According to the primary sites, NK/T cell lymphoma can be divided into nasal and nasal-type. In the early stages of NK/T cell lymphoma, the clinical manifestations are atypical. Nasal NK/T cell lymphoma mainly affects facial midline such as nasal cavity, presents with a neoplasma in the nasal cavity or facial anomaly. Nasal type NK/T cell lymphoma frequently involves skin or gastrointestinal tract, its clinical presentation varies depending on the site of involvement. NK/T cell lymphoma rarely involves bone marrow, usually without involvement of local lymph node. The diagnosis of NK/T-cell lymphoma depends mainly on the clinical manifestations, pathological characteristics, immunological markers of NK or T cell, cytotoxic granule-associated proteins and EBER detection. The important diagnostic conditions are as follows:(1) The patient may occur a neoplasma of nasal cavity or symptoms and signs of involved sites such as skin lumps, ulcers; (2) soakage of abnormal lymphoid cells can be found in pathological examination; (3) NK cell markers (CD56, cytoplasmic CD3, CD2) or T cell markers (CD45RO, CD43, surface CD3) are positive; (4) cytotoxic granule-associated proteins (TIA-1, granzyme B and perforin) are positive; (5) EBER is positive. Optimum treatment for NK/T cell lymphoma is in the exploring. Current studies suggest that radiotherapy is a better treatment for patients with early nasal NK/T cell lymphoma; chemotherapy is the mainstay for patients with nasal type or advanced nasal NK/T cell lymphoma and the efficacies of L-asparaginase-based regimens are better; chemoradiotherapis are adopted more frequently; and hematopoietic stem cell transplantation (HSCT) is an important alternative for NK/T cell lymphomas.