Aggressive natural killer cell leukemia (ANKL) is a rare hematological malignancy, with only a few cases reported in the literature. The overall median survival is estimated to be less than 2 months. Moreover, Epstein–Barr virus (EBV)-negative ANKL is a rarity, with only a few being EBV-negative. We present a 27-year-old male with pancytopenia, massive splenomegaly, and no lymphadenopathy. Bone marrow (BM) aspirate flow cytometry revealed abnormal lymphocytes showing moderate expression of CD56, CD16, CD38, CD2, and CD45, dim expression of CD7 and CD117, and variable cytoplasmic CD3 and were negative for immaturity markers and other T lymphocytic, myeloid, and plasmacytoid dendritic cell markers. The BM biopsy revealed negative terminal deoxynucleotidyl transferase, confirming the diagnosis of ANKL. EBV was negative; hence, the final diagnosis of EBV-negative ANKL was offered. The patient had a progressive course despite two cycles of modified SMILE regimen and CHOP. The addition of daratumumab (anti-CD38 monoclonal antibody) to CHOP resulted in disease remission. The patient has survived for more than 18 months after diagnosis and is presently on follow-up on maintenance daratumumab, awaiting an allogenic stem cell transplant. This article aims to report rare refractory EBV-negative ANKL patients successfully managed with an unconventional regimen; hence, further studies are warranted for targeted therapy in fulminant diseases like ANKL.

Background: Metastasis to regional cervical lymph nodes is an important prognostic factor for oral-cavity squamous cell carcinomas (OSCC). However, it is difficult to predict nodal metastasis in OSCC. The current study was done with an aim to find the association of various clinicopathological factors to the presence of cervical lymph node metastasis in OSCC. Materials and Methods: A retrospective analysis of hospital records from January 2020 to June 2023 was conducted in a tertiary care center. The records of 65 patients were included as per the inclusion criteria. The data were divided into two groups based on the presence or absence of neck nodal metastasis. Various factors such as tumor size, volume, depth of invasion (DOI), lymphovascular invasion, and perineural invasion were statistically compared between the study groups. Results: The oral tongue was the most common subsite (25, 38.5%), followed by buccal mucosa (18, 27.7%). The mean DOI in the group with cervical lymph node metastasis was 9.83 ± 4.83 mm, and it was significantly higher than the group without cervical lymph node metastasis (6.15 ± 4.20 mm, P = 0.004). Conclusion: DOI assessment is one of the most important preoperative assessments in the clinicoradiological assessment of OSCC cases. Further, it is recommended that in cases with DOI >5 mm, the intraoperative frozen section on the nodes dissected in elective neck dissection is done for further determination of the extent of neck dissection.