Aggressive natural killer cell lymphoma/leukemia

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Aggressive natural killer (NK) cell lymphoma/leukemia is a rare and aggressive hematologic malignancy.
• Early diagnosis and understanding of its characteristics are crucial for patient outcomes.

Observation:

• A 70-year-old woman presented with fever, enlarged liver and spleen, retroperitoneal lymphadenopathy, and elevated CA19-9.
• Neoplastic NK cells were detected in peripheral blood and retroperitoneal lymph node biopsy.
• Epstein-Barr virus-associated RNA (EBER) in situ hybridization was positive in neoplastic cells.

Findings:

• Flow cytometry and immunohistochemistry confirmed the phenotype of neoplastic NK cells.
• The patient was diagnosed with aggressive NK cell lymphoma/leukemia.
• The patient had a rapid clinical course, succumbing to the disease 4 days post-diagnosis.

Implications:

• This case underscores the importance of considering aggressive NK cell lymphoma/leukemia in the differential diagnosis of elderly patients with unexplained systemic symptoms and elevated tumor markers.
• The positive EBER in situ hybridization suggests a potential role for Epstein-Barr virus in the pathogenesis of this aggressive NK cell neoplasm.
• Further research into the molecular mechanisms and therapeutic strategies for EBV-associated aggressive NK cell neoplasms is warranted.