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Published on: July 21, 2016
Aggressive granular lymphocyte leukemia of natural killer cell type in an elderly patient
N Ichikawa1, K Kitano, M Higuchi
1Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto.
Insights
A rare case of granular lymphocyte (GL) proliferation, identified as natural killer cells, was observed in a 75-year-old man. This condition, linked to a chromosome 9 inversion, led to organ failure and death.
Area of Science:
- Hematology
- Immunology
- Cytogenetics
Background:
- Granular lymphocyte (GL) proliferation can be associated with various clinical conditions.
- Natural killer (NK) cells are a crucial component of the innate immune system.
Observation:
- A 75-year-old male presented with intermittent fever and peripheral blood findings of GL proliferation.
- Immunophenotyping confirmed the GLs to be of natural killer cell lineage.
- Chromosomal analysis revealed an abnormal monoclonal proliferation of GLs with a unique chromosome 9 inversion.
Findings:
- The patient developed progressive hepatosplenomegaly, icterus, and renal failure.
- Despite chemotherapy, the patient succumbed to respiratory failure.
- Autopsy confirmed disseminated intravascular coagulation and GL infiltration in multiple organs.
Implications:
- This case highlights a rare presentation of NK cell leukemia/lymphoma.
- The unusual chromosome 9 breakpoint may be a specific marker for this condition.
- Further research is needed to understand the pathogenesis and treatment of GL proliferation.
Abstract:
A 75-year-old man was admitted to our hospital because of intermittent fever. His peripheral blood picture showed granular lymphocyte (GL) proliferation. The GLs were immunologically and functionally phenotyped as natural killer cells. Chromosomal analysis of peripheral lymphocytes with interleukin-2 stimulation revealed an inversion of chromosome 9 with an unusual breakpoint, showing abnormal monoclonal proliferation of the GLs. Progressive increase of GL count and hepatosplenomegaly necessitated the start of combined chemotherapy. His condition was complicated by icterus and renal failure, and he died finally of respiratory failure. Autopsy revealed disseminated intravascular coagulation and infiltration of GLs in the bone marrow, spleen, and liver.
