Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia
Objective This paper aims to analyze and summarize the clinical and laboratory characteristics of patients with T-cell large granular lymphocytic leukemia (T-LGLL) and explore the diagnosis and treatment of T-LGLL. Methods A retrospective analysis was conducted on the clinical data of 6 T-LGLL patie...
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Editorial Office of Journal of Diagnostics Concepts & Practice
2024-12-01
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| Series: | Zhenduanxue lilun yu shijian |
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| Online Access: | https://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1740725520748-1001541181.pdf |
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| author | LU Hongyu, LIU Hong, SONG Luxi |
| author_facet | LU Hongyu, LIU Hong, SONG Luxi |
| author_sort | LU Hongyu, LIU Hong, SONG Luxi |
| collection | DOAJ |
| description | Objective This paper aims to analyze and summarize the clinical and laboratory characteristics of patients with T-cell large granular lymphocytic leukemia (T-LGLL) and explore the diagnosis and treatment of T-LGLL. Methods A retrospective analysis was conducted on the clinical data of 6 T-LGLL patients treated at our hospital from March 2019 to December 2022. The cell morphology, bone marrow cell immunophenotyping, genetic testing results, and treatment plans were analyzed and summarized, with follow-up conducted. Results The median age at diagnosis of the 6 T-LGLL patients was 60 (range 54-70) years. All 6 patients presented with anemia at the time of consultation, with 3 requi-ring blood transfusion, 3 having splenomegaly, and 1 having lymphadenopathy. Peripheral blood LGL morphology was typical in all 6 cases, but with low absolute counts. The median count was 1.0 (range 0.4-1.4) × 109/L. Bone marrow cell immunophenotyping showed that all patients’ LGL cells originated from post-thymic mature T cells. 4 patients expressed the common CD3+CD8+CD57+ effector T-cell markers, while 2 expressed the rare CD3+CD8+CD57- memory T-cell markers. Genetic testing revealed monoclonal fragments in the T cell receptor (TCR) of all 6 patients, supporting the clonal abnormality. The next generation gene sequencing results showed STAT3 mutations in 4 of the 6 patients. All 6 patients received immunosuppressive therapy, and follow-up revealed that 5 patients responded to the treatment and 5 out of 6 patients achieved continuous hematological remission. Conclusions The diagnosis of T-LGLL cannot be accurately and early made solely based on typical cell morphology and absolute LGL counts. Additionally, there are significant variations in LGL immunophenotypes. Therefore, an integrated multi-parameter diagnostic approach combining morphology, immunophenotyping, TCR clonal analysis, and molecular biology data from next-generation sequencing is recommended. Currently, immunosuppressive therapy shows good treatment response. |
| format | Article |
| id | doaj-art-32db4d1ac9c848a1bb2ec08d246b975c |
| institution | Kabale University |
| issn | 1671-2870 |
| language | zho |
| publishDate | 2024-12-01 |
| publisher | Editorial Office of Journal of Diagnostics Concepts & Practice |
| record_format | Article |
| series | Zhenduanxue lilun yu shijian |
| spelling | doaj-art-32db4d1ac9c848a1bb2ec08d246b975c2025-08-20T03:49:07ZzhoEditorial Office of Journal of Diagnostics Concepts & PracticeZhenduanxue lilun yu shijian1671-28702024-12-01230661261810.16150/j.1671-2870.2024.06.008Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemiaLU Hongyu, LIU Hong, SONG Luxi01. Department of Hematology,Yangpu Hospital, School of Medicine,Tongji University, Shanghai 200090， China;2. Department of Hematology, Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200233, ChinaObjective This paper aims to analyze and summarize the clinical and laboratory characteristics of patients with T-cell large granular lymphocytic leukemia (T-LGLL) and explore the diagnosis and treatment of T-LGLL. Methods A retrospective analysis was conducted on the clinical data of 6 T-LGLL patients treated at our hospital from March 2019 to December 2022. The cell morphology, bone marrow cell immunophenotyping, genetic testing results, and treatment plans were analyzed and summarized, with follow-up conducted. Results The median age at diagnosis of the 6 T-LGLL patients was 60 (range 54-70) years. All 6 patients presented with anemia at the time of consultation, with 3 requi-ring blood transfusion, 3 having splenomegaly, and 1 having lymphadenopathy. Peripheral blood LGL morphology was typical in all 6 cases, but with low absolute counts. The median count was 1.0 (range 0.4-1.4) × 109/L. Bone marrow cell immunophenotyping showed that all patients’ LGL cells originated from post-thymic mature T cells. 4 patients expressed the common CD3+CD8+CD57+ effector T-cell markers, while 2 expressed the rare CD3+CD8+CD57- memory T-cell markers. Genetic testing revealed monoclonal fragments in the T cell receptor (TCR) of all 6 patients, supporting the clonal abnormality. The next generation gene sequencing results showed STAT3 mutations in 4 of the 6 patients. All 6 patients received immunosuppressive therapy, and follow-up revealed that 5 patients responded to the treatment and 5 out of 6 patients achieved continuous hematological remission. Conclusions The diagnosis of T-LGLL cannot be accurately and early made solely based on typical cell morphology and absolute LGL counts. Additionally, there are significant variations in LGL immunophenotypes. Therefore, an integrated multi-parameter diagnostic approach combining morphology, immunophenotyping, TCR clonal analysis, and molecular biology data from next-generation sequencing is recommended. Currently, immunosuppressive therapy shows good treatment response.https://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1740725520748-1001541181.pdf|leukemia|large granular lymphocytic|lymphocytes|immunophenotyping|immunosuppressive therapy |
| spellingShingle | LU Hongyu, LIU Hong, SONG Luxi Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia Zhenduanxue lilun yu shijian |leukemia|large granular lymphocytic|lymphocytes|immunophenotyping|immunosuppressive therapy |
| title | Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia |
| title_full | Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia |
| title_fullStr | Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia |
| title_full_unstemmed | Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia |
| title_short | Analysis of clinical and laboratory characteristics of six cases with T-cell large granular lymphocytic leukemia |
| title_sort | analysis of clinical and laboratory characteristics of six cases with t cell large granular lymphocytic leukemia |
| topic | |leukemia|large granular lymphocytic|lymphocytes|immunophenotyping|immunosuppressive therapy |
| url | https://www.qk.sjtu.edu.cn/jdcp/fileup/1671-2870/PDF/1740725520748-1001541181.pdf |
| work_keys_str_mv | AT luhongyuliuhongsongluxi analysisofclinicalandlaboratorycharacteristicsofsixcaseswithtcelllargegranularlymphocyticleukemia |