NKTCL, Extranodal Natural Killer T-Cell Lymphoma

In China 40-50% der peripheren T-Zellymphome

Upper Aerodigestive Tract - Natural Killer Cell Lymphoma

Klinische Formen

Feature	Nasal	Extranasal UADT	Extra-UADT
Primary locations	Nasal cavity with or without extension into adjacent structures such as paranasal sinuses	Waldeyer ring (nasopharynx, tonsil, oropharynx, base of the tongue), hypopharynx, larynx, and oral cavity	Skin, gastrointestinal tract, soft tissue, testis, etc;. accounts for 10%-30% of all cases
Immunophenotype	High expression of EBV (90%), CD56, and Ki-67; all cases express at least 1 cytotoxic protein	High expression of EBV (90%); CD56 expression is less common than nasal variant; low proliferation index (Ki-67)	EBV expression is relatively diverse (40%-100%); high proliferation index
Age	Usually adults, median age of 40-50 y	Usually adults, median age of 38-50 y	Usually adults, median age of 50 y
Sex	Male predominance, M:F Z 2-4:1	Male predominance, M:F Z 2.6:1	Male predominance, M:F Z 1.5-2.3:1
Ann Arbor stage	Usually present with early-stage disease, majority with stage I (60%-80%); less common with stage III and IV (10%-25%).	Usually present with early-stage disease, stage I < 20%, stage II 50%-60%; more advanced- stage disease (20%-30%)	Usually present with disseminated and advanced- stage disease ( > 50%)
Performance status	Good	Good	Poor, frequently ECOG  2
Elevated LDH	Frequency (20%-50%)	Frequency (20%-50%)	High frequency (50%-70%)
Lymph node involvement	Low frequency of lymph node involvement at diagnosis ( < 20%)	Frequent involvement of cervical lymph node ( > 50%)	High frequency of regional lymph node involvement
IPI	Usually low risk, IPI 0-1 > 90%	Usually low risk, IPI 0-1  80%	Usually high risk, IPI 0-1 25%-58%
Failure patterns	Extranodal organs; skin is the most common site	Lymph nodes and extranodal organs	Extranodal organs
Clinical course	Aggressive	Aggressive	Highly aggressive
Prognosis	Favorable outcome in stage I patients treated with appropriate radiation therapy; poor for stage II-IV patients	Relatively favorable outcome compared with nasal or extra- UADT variants	Extremely poor prognosis; median survival 3-20 months

Modifiziert nach (4)

Quellen

1.) Sun J, Yang Q, Lu Z, et al.:
Distribution of lymphoid neoplasms in China: Analysis of 4,638 cases according to the World Health Organization classification.
Am J Clin Pathol 138(2012):429-434.

2. Au WY, Weisenburger DD, Intragumtornchai T, et al.:
Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: A study of 136 cases from the International Peripheral T-Cell Lymphoma Project.
Blood 113(2009):3931-3937.

3. Yang QP, Zhang WY, Yu JB, et al. :
Subtype distribution of lymphomas in Southwest China: Analysis of 6,382 cases using WHO classification in a single institution.
Diagn Pathol 6(2011):77

4.) Liu Q-F, et al.:
Immunophenotypic and Clinical Differences Between the Nasal and Extranasal Subtypes of Upper Aerodigestive Tract Natural Killer/T-Cell Lymphoma.
Int J Radiation Oncol Biol Phys 88(2014):806-813