[Primary nasopharyngeal lymphoma with CD3- and CD56+ phenotype]

Area of Science:

• Oncology
• Immunology
• Hematology

Background:

• A 73-year-old male presented with nasal hemorrhage, high-grade fever, and a nasopharyngeal tumor.
• Physical examination revealed generalized skin eruptions, subcutaneous tumors, splenomegaly, and lymphadenopathy.

Observation:

• Biopsies confirmed non-Hodgkin's lymphoma (Diffuse small cleaved type) in the nasopharyngeal and cutaneous lesions.
• Peripheral blood showed 10% blastoid cells, while bone marrow revealed 60% blastoid cells with characteristic morphology.
• Flow cytometry identified blastoid cells expressing CD56 (NKH-1) and Ia, but lacking T-cell, B-cell, and myeloid antigens.

Findings:

• The phenotype of subcutaneous tumor cells mirrored the blastoid cells in the bone marrow.
• Absence of T-cell receptor gene rearrangements in blastoid cells further supported a non-T, non-B lineage.
• The patient was treated with radiotherapy and chemotherapy but succumbed to disseminated intravascular coagulation (DIC) with pulmonary bleeding.

Implications:

• These findings suggest that the nasopharyngeal lymphoma originated from Natural Killer (NK) cells.
• This case highlights the importance of immunophenotyping in diagnosing rare lymphoid malignancies.
• Understanding NK-cell lymphomas is crucial for developing targeted therapeutic strategies.