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[Large granular lymphocyte leukemia]
Chao Xiao1, Xi Zhang1, Chun-Kang Chang1
1Department of Hematology, Affiliated Sixth People Hospital, Shanghai Jiaotong University, Shanghai 200233, China.
Insights
Large granular lymphocytic (LGL) leukemia encompasses T-cell LGL leukemia and NK-cell disorders. Treatment ranges from watchful waiting to intensive chemotherapy and stem cell transplant, depending on disease subtype and severity.
Area of Science:
- Hematology
- Oncology
- Immunology
Context:
- Large granular lymphocytic (LGL) leukemia is a rare clonal lymphoproliferative disorder.
- WHO classification identifies T-cell LGL leukemia (T-LGL) and chronic lymphoproliferative disorders of NK-cells (CLPD-NK).
- Significant overlap exists between T-LGL and CLPD-NK in clinical presentation and management.
Purpose:
- To review the pathogenesis, diagnosis, treatment, and prognosis of LGL leukemia subtypes.
- To discuss current management strategies based on disease characteristics.
- To highlight the challenges posed by the rarity of these conditions.
Summary:
- LGL leukemia includes T-LGL, CLPD-NK, and aggressive NK-cell leukemia.
- Asymptomatic patients often require no immediate treatment; immunosuppressive therapy is used for cytopenias.
- Aggressive forms necessitate intensive chemotherapy and stem cell transplantation.
Impact:
- Provides a comprehensive overview for clinicians managing LGL leukemia.
- Aids in understanding the distinct clinical courses and treatment approaches for different LGL subtypes.
- Highlights the need for further research and clinical trials due to the rarity of these leukemias.
Abstract:
Large granular lymphocytic (LGL) leukaemia is an uncommon clonal lymphoproliferative disorder. The WHO classification recognizes three distinct disorders of LGLs: T-cell large granular lymphocytic leukaemia (T-LGL), chronic lymphoproliferative disorders of NK-cells (CLPD-NK) and aggressive NK-cell leukaemia. Despite the different origin of cells, there is considerable overlap between T-LGL and CLPD-NK in terms of clinical presentation and treatment. Majority of these patients are asymptomatic and may not need treatment. When significant cytopenias occur, the application of immunosuppressive therapy often should be considered. In contrast, aggressive NK cell leukemia and the rare CD56(+) aggressive T-LGL leukemia have a fulminant clinical course and an earlier age of onset, therefore, more intensive combination chemotherapy is required, followed by allogeneic hematopoietic stem cell transplantation. However, these diseases are relatively rare, there are few clinical trials to guide management. In this review, the pathogenesis, diagnosis, treatment and prognosis of this leukemia are summarized and discussed.
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