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Unique T-helper leukaemia with cytoplasmic granules and convoluted nuclei
M Watanabe1, Y Shimamoto, M Sano
1Department of Internal Medicine, Saga Medical School, Japan.
Insights
A rare case of CD4+ helper T-cell leukemia presented with unusual cell morphology, distinct from typical CD8+ large granular lymphocytes. This finding highlights the diverse presentations of T-cell leukemias.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- CD4+ helper T-cell lymphocytic leukemia is a rare hematologic malignancy.
- Morphological variations in T-cell leukemias contribute to diagnostic challenges.
Observation:
- An unusual case of CD4+ T-cell leukemia was observed in a 67-year-old Japanese woman.
- The leukemia cells exhibited convoluted nuclei and dense cytoplasmic granules, atypical features for CD4+ cells, resembling CD8+ large granular lymphocytes.
- Human T-lymphotropic virus type I (HTLV-I) infection was ruled out via serology and molecular analysis.
Findings:
- Monoclonal rearrangement of the TCR-beta chain gene confirmed the neoplastic nature of the T-cells.
- The patient presented with elevated lactate dehydrogenase (LDH) levels but normal serum calcium.
- Absence of skin involvement and splenomegaly was noted.
Implications:
- This case underscores the morphological heterogeneity within CD4+ T-cell leukemias.
- It emphasizes the importance of comprehensive immunophenotypic and genotypic analysis for accurate diagnosis and classification of T-cell malignancies.
- Understanding these variations is crucial for advancing the diagnosis and treatment strategies for T-cell lymphoid leukaemias.
Abstract:
An unusual case of CD4+ helper T-cell lymphocytic leukaemia is reported in a 67-year-old Japanese woman. CD4+ cells showed convoluted nuclei and dense cytoplasmic granules, features usually present in CD8+ large granular lymphocytes and disorders of this particular cell type. Serum did not show antibodies to HTLV-I and HTLV-I proviral DNA integration was not evident by Southern blot analysis or after PCR. A monoclonal rearrangement of the TCR-beta chain gene was evident when hybridization methods were used. The patient died 11 months after diagnosis. No skin involvement, or splenomegaly was evident. Serum LDH levels were markedly elevated but serum calcium levels were within normal limits. The case is discussed and compared to other T-cell lymphoid leukaemias. The heterogeneity in the morphology of CD4+ T cell leukaemias is stressed.