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Aggressive natural killer cell lymphoma/leukemia. A recently recognized clinicopathologic entity
1Department of Laboratories, North Shore University Hospital-Cornell University Medical College, Manhasset, NY 11030.
The American Journal of Surgical Pathology
|December 1, 1993
Summary
Aggressive natural killer cell lymphoma/leukemia is a rapidly progressive cancer. This case highlights its unique presentation as testicular lymphoma, a rare and challenging diagnosis.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Aggressive natural killer (NK) cell lymphoma/leukemia is a rare hematologic malignancy.
- It typically affects young males and presents with rapid progression, lymphadenopathy, hepatosplenomegaly, and bone marrow involvement.
Observation:
- This case presented uniquely as testicular lymphoma.
- Diagnostic clues included cytoplasmic granules in tumor cells and loss of T-cell antigens.
- Immunophenotyping confirmed NK cell markers (CD16, CD56, CD57) and negativity for T-cell and B-cell markers.
Findings:
- Genotyping revealed germline configuration for immunoglobulin and T-cell receptor genes.
- Polymerase chain reaction did not detect human T-cell leukemia virus type I/II.
- The disease demonstrated resistance to available chemotherapeutic regimens.
Implications:
- Recognizing this aggressive entity is crucial for timely diagnosis and management.
- The testicular presentation is a novel finding for aggressive NK cell lymphoma/leukemia.
- Further research is needed to develop effective therapeutic strategies for this aggressive malignancy.