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Published on: March 30, 2018
Blastic NK-cell lymphoma/leukemia with T-cell receptor gamma rearrangement
1Department of Clinical Hematology, Osaka City University Medical School, 1-4-3, Asahimachi, Abeno-ku, Osaka-shi, Osaka, 545-8586 Japan.
Insights
This study reports a rare case of T-cell lymphoblastic leukemia in a 79-year-old man. The aggressive leukemia showed a poor prognosis, relapsing early despite initial chemotherapy.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- T-cell acute lymphoblastic leukemia (T-ALL) is a rare and aggressive hematologic malignancy.
- Accurate immunophenotypic and genotypic characterization is crucial for diagnosis and classification.
Observation:
- A 79-year-old Japanese male presented with dyspnea, exanthema, hepatosplenomegaly, and lymphadenopathy.
- Peripheral blood revealed abnormal lymphoblastic cells with specific immunophenotypic markers (CD2+, CD4+, CD56+, HLA-DR+).
- Genotypic analysis showed T cell receptor (TCR) gamma chain rearrangement exclusively.
Findings:
- The patient was diagnosed with a rare T-ALL subtype.
- Despite initial positive response to chemotherapy, the disease exhibited early relapse.
- The patient succumbed to the disease within 6 months of diagnosis.
Implications:
- This case highlights the aggressive nature and poor prognosis of certain T-ALL subtypes, even with treatment.
- Emphasizes the importance of comprehensive immunophenotypic and genotypic analysis for diagnosing and managing rare hematologic malignancies.
- Contributes to understanding the clinical spectrum and outcomes of T-cell lymphoblastic leukemia.
Abstract:
A 79-year-old Japanese man was admitted to our hospital with dyspnea in June 1999. Physical examination revealed general exanthema, hepatosplenomegaly, and lymphadenopathy. Increased numbers of abnormal cells were observed in peripheral blood; these cells were of lymphoblastic morphology with high nuclear/cytoplasm ratios and few azurophilic granules. Immunophenotypic analysis revealed positivity for CD2, CD4, CD56, and HLA-DR, and negativity for CD3, CD13, CD16, CD33, CD34, and T cell receptor (TCR). On genotypic analysis, TCRgamma chain was rearranged, but neither the TCRbeta chain nor TCRdelta chain. Despite an initial good response to chemotherapy the disease relapsed in the early stage, and the patient died 6 months after diagnosis.
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