Gamma-delta T Cell Large Granular Lymphocyte Leukaemia With Multiple Cutaneous Nodules That Showed Spontaneous Regression
Suzuki, C., Hirai, I., Nomura, H., Ouchi, T., Okayama, M., Okamoto, S., Amagai, M., Tanese, K., & Takahashi, H. (2019). Gamma-delta T cell large granular lymphocyte leukaemia with multiple cutaneous nodules that showed spontaneous regression. Journal of the European Academy of Dermatology and Venereology : JEADV, 33(3), e134–e137. https://doi.org/10.1111/jdv.15341
View Original Source →Abstract
T cell large granular lymphocyte leukemia (T-LGLL) is a rare chronic lymphoproliferative disorder of mature post-thymic large granular lymphocytes (LGL); either cytotoxic T-lymphocytes or natural killer cells.1 Major phenotype of LGL cells is TCRαβ+CD3+CD4-CD5+CD8+CD27-CD28-CD45-CD57+.2 Less than 10% of cases express TCRγδ instead of TCRαβ.2 Most cases of T-LGLL have an indolent course1 and its cutaneous manifestations have been poorly documented. This article is protected by copyright. All rights reserved.
Case Details
Disease Location
Blood, skin
Personal Characteristics
47-year-old japanese woman, 8 year history of increased number of atypical lymphocytes in the peripheral blood
Clinical Characteristics
Peripheral blood smear and bone marrow biopsy revealed proliferation of large granular lymphocytes. Flow cytometry detected prominent proliferation of CD3+,CD4-,CD8-, tcr gamma-delta+ cells. The diagnosis of gamma-delta t cell large granular lymphocyte leukaemia (t- lgll) was made. Multi-centric cutaneous nodules started appearing on the scalp, face, shoulder, and abdomen 6 years after the diagnosis. Even after one nodule disappeared spontaneously, another nodule appeared at a different site.skin biopsy revealed predominance of CD3+,CD4-,CD8-, tcr gamma-delta + cells, suggesting that the nodules consisted of cutaneous infiltration of t-lgll cells
Remission Characteristics
Some nodules disappeared spontaneously. In the specimen of the clinically regressing nodule, a massive necrotic area was noted in the centre of the lesion. CD4+ and CD8+ t cells were observed within the necrotic area as well.
Treatment & Mechanisms
Proposed Remission Mechanisms
CD4+ and CD8+ t cell infiltration may have had a cytotoxic effect on the leukaemic cells, and resulted in tumour regression
Clinical Treatment
Biopsy
Non-Clinical Treatment
None reported