Extra-nasal NK/T-cell lymphoma: A rare case with a rarer presentation

Area of Science:

• Oncology
• Immunology
• Pathology

Background:

• Extranodal (NK/T) cell lymphoma (NKTCL) of nasal type (ENKL) typically affects the nasal and upper airway regions.
• This lymphoma subtype is associated with Epstein-Barr virus (EBV) and can rarely present with extranodal involvement in various organs or as disseminated disease.

Observation:

• A 65-year-old male presented with bilateral orbital swelling, right parotid swelling, fever, mediastinal lymphadenopathy, pleural effusion, and hepatomegaly.
• Pleural fluid cytology revealed monomorphic lymphoid cells with characteristic morphology and hemophagocytosis by histiocytes.
• Flow cytometry identified abnormal lymphoid cells positive for CD45, CD2, and CD56, while histopathology of orbital tissue confirmed atypical lymphoid cells positive for CD56.

Findings:

• Morphological and immunophenotypic analyses supported a diagnosis of NKTCL, extra-nasal type.
• The patient's condition was aggressive and did not respond to intensive therapy, leading to a fatal outcome.

Implications:

• This case underscores the importance of considering extranodal NK/T-cell lymphoma in the differential diagnosis of unusual presentations involving extranodal sites.
• The findings emphasize the aggressive nature of extra-nasal NKTCL and the challenges in managing disseminated forms of the disease.
• Further research into early diagnostic markers and novel therapeutic strategies for extra-nasal NKTCL is warranted.