CHARACTERIZATION OF THE ONCOGENIC PHENOTYPE IN EXTRANODAL NATURAL KILLER/T‐CELL LYMPHOMA, NASAL TYPE THROUGH GENE EXPRESSION PROFILE. SPANISH LYMPHOMA GROUP GELTAMO

Introducción: El linfoma de células T/Natural Killer (T/NK) extranodal, tipo nasal, es un linfoma extra-ganglionar poco frecuente, con extensión a lo largo de la línea media facial, rápidamente progresivo, catastrófico y de mal pronóstico, por lo que también se le conoce como “granuloma letal de la línea media”. Objetivo: El propósito de este artículo es revisar la literatura disponible y actualizada sobre el linfoma de células T/NK extranodal, tipo nasal: manifestaciones clínicas, estándares de enfoque, diagnóstico, pronóstico y tratamiento. Diseño: Revisión Narrativa de la literatura. Metodología: Revisión de la literatura mediante búsqueda selectiva por términos MeSH: Extranodal Natural Killer /T cell lymphoma, nasal type, lethal midline granuloma, de las bases de datos: MEDLINE, Current Contents, Cochrane, Pubmed y Scielo, entre los años 2000 y 2014. Resultados: Se revisaron 36 artículos según los requerimientos de los objetivos. El linfoma de células T/NK es una neoplasia rápidamente progresiva, destructiva y de mal pronóstico excepto en los casos donde el diagnóstico ha sido oportuno. Conclusiones: El diagnóstico y tratamiento tempranos del linfoma de células T/NK extranodal, tipo nasal, son las únicas herramientas para mejorar el mal pronóstico y gran afectación en la calidad de vida de los pacientes con esta enfermedad.

Download Full-text

Daratumumab monotherapy for patients with relapsed or refractory natural killer/T-cell lymphoma, nasal type: an open-label, single-arm, multicenter, phase 2 study

Journal of Hematology & Oncology ◽

10.1186/s13045-020-01020-y ◽

2021 ◽

Vol 14 (1) ◽

Author(s):

Huiqiang Huang ◽

Jun Zhu ◽

Ming Yao ◽

Tae Min Kim ◽

Dok Hyun Yoon ◽

...

Keyword(s):

T Cell ◽

Natural Killer ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Phase 2 ◽

Nasal Type ◽

Natural Killer T Cell ◽

Phase 2 Study ◽

Duration Of Response ◽

Killer T Cell

Abstract Background Natural killer/T-cell lymphoma (NKTCL) is a disease with limited treatment options and poor outcomes. Daratumumab monotherapy demonstrated clinical activity in a single-patient case report. We present data from the primary analysis of a phase 2 study of daratumumab monotherapy in relapsed or refractory (R/R) NKTCL. Methods This phase 2 study with Simon’s two-stage design evaluated daratumumab in patients with histologically confirmed extranodal NKTCL, nasal type, per WHO classification that was refractory to or relapsed after ≥ 1 line of chemotherapy, who were not candidates for other treatment modalities. All patients received daratumumab 16 mg/kg intravenously once weekly for Cycles 1 and 2, every other week for Cycles 3 through 6, and every 4 weeks thereafter until progression or unacceptable toxicity; all cycles were 28 days. The primary end point was objective response rate (ORR) based on blinded independent central review per Revised Criteria for Response Assessment of Hodgkin and non-Hodgkin Lymphoma (Lugano classification). Results In total, 32 Asian patients received daratumumab. The ORR was 25.0% (95% confidence interval [CI] 11.5–43.4); all 8 responders had a partial response; and the median duration of response was 55.0 days (95% CI 29–339). At 10.2 months of median follow-up, median progression-free survival (PFS) was 53.0 days (95% CI 43–106); the 4-month PFS rate was 13.0%. Median overall survival (OS) was 141.0 days (95% CI 94–438); the 6-month OS rate was 42.9%. Nineteen (59.4%) patients had grade 3/4 treatment-emergent adverse events (TEAEs); the most common was thrombocytopenia (25.0%; n = 8). TEAEs leading to death occurred in 4 patients (death, respiratory failure, septic shock, and pneumonia); all were unrelated to daratumumab. Conclusions In patients with R/R NKTCL, daratumumab monotherapy was well tolerated with no new safety concerns and achieved an ORR of 25.0%. However, no patients achieved complete response, and duration of response was short. Trial registration ClinicalTrials.gov, NCT02927925. Registered 7 October 2016.

Download Full-text

Salvage radiotherapy improves survival in patients with locoregionally relapsed stage IE–IIE extranodal natural killer/T-cell lymphoma, nasal type

Therapeutics and Clinical Risk Management ◽

10.2147/tcrm.s164376 ◽

2018 ◽

Vol Volume 14 ◽

pp. 1083-1090

Author(s):

Qin Tong ◽

Shuming Ouyang ◽

Lingling Feng ◽

Hanyu Wang ◽

Yunfei Xia ◽

...

Keyword(s):

T Cell ◽

Natural Killer ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Salvage Radiotherapy ◽

Nasal Type ◽

Natural Killer T Cell ◽

Killer T Cell ◽

Natural Killer T

Download Full-text

An Unusual Case of Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Masquerading as Dacryocystitis and Sinusitis

Ear Nose & Throat Journal ◽

10.1177/0145561320987643 ◽

2021 ◽

pp. 014556132098764

Author(s):

Anthony N. Eze ◽

Adedoyin O. Kalejaiye

Keyword(s):

T Cell ◽

Natural Killer ◽

Cell Lymphoma ◽

Central Nervous System Involvement ◽

The United States ◽

T Cell Lymphoma ◽

Nasal Type ◽

Natural Killer T Cell ◽

Killer T Cell ◽

Natural Killer T

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) is a rare and highly aggressive non-Hodgkin lymphoma originating from NK or γδ T cells infected by Epstein-Barr virus (EBV). In the United States, NNKTL is usually noted in people of Asian or Hispanic descent. Natural killer/T-cell lymphoma, nasal type commonly involves the upper aerodigestive tract, including the nasopharynx, nasal cavity, Waldeyer’s ring, and oropharynx. Extensive local destruction and invasion has been noted, especially of the paranasal sinuses, hard palate, and central nervous system; involvement of the nasolacrimal duct with dacryocystitis is yet to be reported. We report a rare case of a Hispanic man with extranodal NNKTL masquerading as persistent dacryocystitis and necrotizing sinusitis unresponsive to antibiotics and surgical intervention. An extensive background of necrosis and inflammation was noted on pathology, and additional analysis with immunohistochemistry and in situ hybridization after repeat biopsy were necessary for accurate diagnosis.

Download Full-text