Nasal NK/T-Cell Lymphoma with Disseminated Disease Treated with Aggressive Combined Therapy

Abstract

Thirty-two patients with nasal NK/T-cell lymphoma and disseminated disease (lung, skin, and bone marrow) were treated with an intensive combined therapy that consisted of three cycles of CMED (cyclophosphamide 2 g/m(2), metothrexate 200 mg/m(2), etoposide 600 mg/m(2), and dexamethasone 80 mg/m(2) with leucovorin rescue administered 24 h after) every 14 d, following high-dose radiotherapy: 55 Gy in 20 sesions to centrofacial region and three cycles more of the same chemotherapy regimen. To ameliorate the presence of severe granulocytopenia, granulocyte colony-stimulating factor, 5 microg/kg, daily for 14 d, begun on d 2 after chemotherapy, was administered. Complete response was achieved in 21 cases (65%); failure or progression was observed in 11 cases (35%). With a median follow-up of 69.1 mo, relapse has not been observed; thus, actuarial curves at 5 yr showed that event-free survival (EFS) is 100% in 21 patients and overall survival (OS) is 65%. Granulocytopenia grade IV was observed in 15% cycles, Nonhematological toxicity was mild and well tolerated. Radiotherapy was well tolerated; only mild mucositis was observed. Nasal NK/T-cell lymphoma is an rare presentation of malignant lymphoma (<1% of all cases) with a worse prognosis; less than 5% patients are alive free of disease at 1 yr. The use of intensive more specific chemotherapy and high dose of local radiotherapy, appear to be an excellent therapeutic approach with improvement in EFS and OS.

Current and future management of NK/T-cell lymphoma based on clinical trials

“…First, a Mexican group reported the results of a clinical study for newly diagnosed advanced nasal NKTCL [50]. There are no descriptions of the rationale for the planned sample size in their report.…”

NK cell lymphoma, nasal type, with massive lung involvement: a case report

“…Regardless of the primary location, extranodal NK/T cell lymphoma presents as localized disease in approximately two thirds of patients [6,12]. Local extension of the tumor is more common than distant involvement and regional lymph node spread is a common occurrence [6,18].…”

“…Local extension of the tumor is more common than distant involvement and regional lymph node spread is a common occurrence [6,18]. Dissemination of NK/T cell lymphoma is rare and appears to be organ specific, with the most commonly involved secondary sites being the skin, gastrointestinal tract, liver, bone marrow, and central nervous system [6,9,10,18]. Lung involvement by NK/T cell lymphoma is very rare and most commonly occurs with widely disseminated disease [6,9,10].…”

“…Dissemination of NK/T cell lymphoma is rare and appears to be organ specific, with the most commonly involved secondary sites being the skin, gastrointestinal tract, liver, bone marrow, and central nervous system [6,9,10,18]. Lung involvement by NK/T cell lymphoma is very rare and most commonly occurs with widely disseminated disease [6,9,10]. NK/T cell lymphoma arising in the lung is extremely rare and only a few cases have been reported in the literature [7,8,11].…”

Pediatric Natural Killer Cell Malignancy

“…In a series of 108 patients with early-stage ENKL receiving radiotherapy followed by chemotherapy consisting with cyclophosphamide, epirubicin, vincristine, prednisolone, and bleomycin [Avilés et al, 2003], combined therapy demonstrated high efficacy with a 92% overall response rate and 8-year OS of 86%. In early-stage ENKL, Guo et al reported that patients who received CHOP followed by radiotherapy (45 Gy) had a CR rate of only 49% as compared to 100% for those who received radiotherapy prior to CHOP [Guo et al, 2008].…”