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Updated: Aug 11, 2026
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VDJ-Seq: Deep Sequencing Analysis of Rearranged Immunoglobulin Heavy Chain Gene to Reveal Clonal Evolution Patterns of B Cell Lymphoma
Published on: December 28, 2015
Clonal diseases of large granular lymphocytes
1Veterans Administration Hospital, Syracuse, NY 13210.
Blood
|July 1, 1993
Summary
Large granular lymphocytes (LGLs) can cause distinct clinical syndromes. While T-LGL leukemia involves clonal CD3+ LGLs, most CD3- LGL increases show polyclonal lymphocytosis and a chronic course, requiring further study for clonal progression.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Large granular lymphocytes (LGLs) are involved in various hematologic and immunologic conditions.
- Increased LGL counts can manifest as distinct clinical syndromes, including T-cell large granular lymphocyte (T-LGL) leukemia and natural killer (NK)-LGL leukemia.
- Understanding the nature of LGL proliferation is crucial for diagnosis and management.
Purpose of the Study:
- To differentiate between distinct clinical syndromes associated with increased circulating LGLs.
- To characterize the immunophenotypic and clinical features of T-LGL leukemia and NK-LGL leukemia.
- To investigate the underlying mechanism of LGL lymphocytosis in patients without overt NK-LGL leukemia.
Main Methods:
- Analysis of clinical presentations and immunophenotypic characteristics (CD3+, CD3-) of circulating LGLs.
- Distinguishing between clonal and polyclonal LGL lymphocytosis using genetic analyses (X-linked gene analyses).
- Correlating LGL proliferation patterns with clinical syndromes and disease course.
Main Results:
- T-LGL leukemia is characterized by clonal CD3+ LGL proliferation, often presenting with chronic neutropenia and autoimmune features.
- NK-LGL leukemia involves clonal CD3- LGL proliferation, typically presenting acutely with hepatosplenomegaly and systemic illness.
- Most patients with increased CD3- LGLs exhibit polyclonal lymphocytosis and a chronic clinical course, without features of NK-LGL leukemia.
Conclusions:
- Circulating LGL increases encompass at least three distinct clinical syndromes.
- While T-LGL and NK-LGL leukemias involve clonal CD3+ and CD3- LGL proliferations respectively, a significant subset of CD3- LGL increases represents polyclonal lymphocytosis with a chronic course.
- Further research is necessary to ascertain the potential for clonal progression in patients with polyclonal LGL lymphocytosis.
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