AUTHOR=Dapkevičiūtė-Purlienė Austėja , Augustinavičius Vytautas , Žučenka Andrius 

TITLE=Case report: Relapsed/refractory extranodal natural killer/T-cell lymphoma nasal type with extensive central nervous system involvement

JOURNAL=Pathology and Oncology Research

VOLUME=Volume 28 - 2022

YEAR=2023

URL=https://www.por-journal.com/journals/pathology-and-oncology-research/articles/10.3389/pore.2022.1610866

DOI=10.3389/pore.2022.1610866

ISSN=1532-2807

ABSTRACT=Background: Extranodal natural killer/T-cell lymphoma (ENKL) is a rare subtype of mature T and natural killer cell lymphomas associated with Epstein-Barr virus.
Case: A 20-year-old presented with severe neurological symptoms and was diagnosed with stage IV ENKL, nasal type, with CNS involvement. Overall, the patient received 9 treatment lines, including chemotherapy, craniospinal irradiation, allogeneic stem cell transplant (alloSCT), donor lymphocyte infusions, and novel agents (Nivolumab, Daratumumab, Thalidomide, Lenalidomide, virus-specific T cells) combined with intrathecal chemotherapy. The treatment effect was evaluated in both blood and CSF (cerebrospinal fluid). First-line SMILE chemotherapy resulted in systemic and CNS remission. Later Cytarabine-based chemotherapy and Daratumumab combination helped to reinduce remission before alloSCT. 
Conclusion: We show that efficacy monitoring should include both blood and CSF analysis. High-dose Cytarabine-based chemotherapy in combination with Daratumumab and intrathecal chemotherapy may be considered as salvage CNS-directed therapies. We add to existing limited data that Daratumumab penetrates the blood-brain barrier.