EBV Positive Aggressive NK-Cell Leukemia

Introduction: Aggressive NK cell leukemia (ANKL) is a rare malignant lymphoproliferative disorder of mature NK cells associated with poor overall survival. Significant variation in the morphology of the neoplastic cells on peripheral smear makes it diagnostically challenging. Case Report: A 54-year old male presented with fever and icterus since 15 days. Peripheral smear showed 64% abnormal cells. Flow cytometry showed positivity of abnormal cells for CD2, CD3, CD7, CD56 and HLA-DR. EBV DNA tested positive by Real-time PCR. A final diagnosis of aggressive NK-cell leukemia was made. Conclusion: The diagnosis of aggressive NK cell leukemia is usually delayed due to the non-specific clinical symptoms and varied morphology. However, early recognition and diagnosis of the disease is important, as the introduction of combined chemotherapy and allogenic hematopoietic stem cell transplantation can help achieve complete response and potential cure.