Related Experiment Videos
[CD3-negative natural killer cell leukemia with aggressive clinical course]
Y Nozawa1, M Miyazawa, K Tasaki
1First Department of Pathology, Fukushima Medical College.
Summary
This study details a rare autopsy case of CD3-negative large granular cell leukemia in a 15-year-old male. The aggressive leukemia, characterized by natural killer cell phenotype, led to fatal hyperkalemia and acidosis despite treatment.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Large granular cell leukemia (LGCL) is a rare hematologic malignancy.
- CD3-negative LGCL with a natural killer (NK) cell phenotype presents unique diagnostic challenges.
Observation:
- A 15-year-old male presented with high fever, cervical lymphadenopathy, and hepatosplenomegaly.
- Peripheral blood showed 45% large granular lymphocytes.
- Immunohistochemistry revealed neoplastic cells expressing CD2, CD38, CD56, and HLA-DR, but lacking CD3, CD4, and CD8.
Findings:
- Southern blot analysis confirmed germline immunoglobulin (Ig) and T cell receptor (TCR) genes.
- The case was diagnosed as CD3-negative large granular cell leukemia with an NK cell phenotype.
- Despite anti-leukemic therapy, the patient succumbed to hyperkalemia and acidosis.
Implications:
- This case highlights the aggressive nature and diagnostic complexities of CD3-negative LGCL.
- Autopsy findings confirmed extensive leukemic infiltration of the liver and spleen.
- Understanding NK-cell LGCL is crucial for developing targeted therapeutic strategies.