[Incidence and treatment of extranodal natural killer/T-cell lymphoma nasal type. Hungarian experiences]. / Nasalis típusú extranodalis natural killer T-sejtes lymphoma hazai elofordulása és kezelésével szerzett tapasztalatok.
Orv Hetil
; 158(41): 1635-1641, 2017 Oct.
Article
en Hu
| MEDLINE
| ID: mdl-29025287
ABSTRACT
INTRODUCTION:
Extranodal natural killer/T (NK/T) cell lymphoma, nasal type (ENKTL) represents a rare subtype of T-cell lymphomas with aggressive clinical behavior according to WHO 2016 classification.AIM:
ENKTL has distinctive geographic distribution with higher incidence in Asia and Latin America (10% of all non-Hodgkin lymphoma cases), than in Europe and North America (<1%). ENKTL tipically origins from nasopharynx and upper aerodigestive tract. Anthracycline-based chemotherapy regimens are largely ineffective in the treatment of ENKTL.METHOD:
Our aims were to evaluate the incidence and treatment strategies of ENKTL patients in Hungarian Haematological Centres between 2003 and 2015. Altogether 20 patients with ENKTL were treated in the 4 haematological hospitals (malefemale ratio 128, with median 49.5 years of age).RESULTS:
Ten patients had localized (stage I-II) disease at the time of the diagnosis. Seventeen patients were treated with chemotherapy (11/CHOP, CHOP-like, 2/HyperCVAD, 1/ProMACECytaBom, 1/SMILE, 2/others), which was completed with involved-field radiation therapy (IFRT) (40-46 Gy) in 6 cases were used. After first-line therapy 9 patients achieved complete remission (CR), 3 patients had partial remission (PR), 3 patients had progressive disease (PD), and 2 patients had stable disease (SD). Median follow-up was 32 (3-113) months. Five patients received second-line therapy for progressive or recurrent disease [2/DHAP, 1/VIM, 1/HyperCVAD, 1/ProMACECytaBom]. None of the patients achieved CR after second-line therapy. Two patients have undergone autologous hematopoietic stem cell transplantation (HSCT) after the first CR.CONCLUSION:
ENKTL treatment is more effective with nonanthracycline-containing regimens. L-asparaginase containing chemotherapy and concurrent or sequential chemo-radiotherapy improves survival and CR rates. Orv Hetil. 2017; 158(41) 1635-1641.Palabras clave
Texto completo:
1
Colección:
01-internacional
Asunto principal:
Neoplasias Nasales
/
Linfoma Extranodal de Células NK-T
Tipo de estudio:
Incidence_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Adult
/
Humans
/
Middle aged
País/Región como asunto:
Europa
Idioma:
Hu
Revista:
Orv Hetil
Año:
2017
Tipo del documento:
Article