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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
Published on: November 26, 2018
[Chronic lymphocytic leukemia with peripheral T lymphocytes expressing CD 2+, CD 3+, CD 4-, CD 8-, CD 16+, and CD 56+
Y Furukawa1, K Tanaka, T Hasuike
1Department of Laboratory Medicine, Osaka City University Medical School.
Insights
This study details a rare case of T-cell large granular lymphocyte (T-LGL) leukemia presenting with thrombocytopenia. The findings suggest a potential shared origin for T-LGL and natural killer (NK) cells.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disorder.
- Distinguishing between T-cell LGL (T-LGL) and natural killer (NK) cell lymphocytosis can be challenging.
- This case explores the immunophenotypic characteristics and clinical course of a patient with suspected T-LGL leukemia.
Observation:
- A 33-year-old male presented with thrombocytopenia and splenomegaly.
- Peripheral blood flow cytometry revealed abnormal lymphocytes expressing CD2, CD3, CD11, CD16, and CD56 (T-LGL).
- Lymph node lymphocytes expressed CD2, CD16, CD38, and CD56, but lacked CD3 (NK cells).
Findings:
- Splenectomy specimen showed infiltration by pleomorphic lymphocytes, consistent with chronic lymphocytic leukemia.
- Post-splenectomy, platelet counts normalized, but lymphocytosis persisted.
- The patient later required chemotherapy for recurrent thrombocytopenia and hepatomegaly, ultimately succumbing to sepsis-induced disseminated intravascular coagulation.
Implications:
- The distinct yet overlapping immunophenotypes of lymphocytes from peripheral blood and lymph nodes suggest a possible common progenitor for T-LGL and NK cells.
- This case highlights the complex clinical presentation and potential diagnostic challenges in LGL lymphoproliferative disorders.
- Further research into the differentiation pathways of LGL and NK cells is warranted.
Abstract:
A 33-year-old man was hospitalized because of thrombocytopenia and severe splenomegaly. On admission 78% of peripheral lymphoid cells were abnormally large, with pale cytoplasm. Flow cytometry of the abnormal lymphocytes showed that they expressed CD 2, CD 3, CD 11, CD 16, and CD 56, but not CD 4 nor CD 8, so they were T-cell large granular lymphocytes (T-LGL). Abnormal lymphocytes obtained from a lymph node expressed CD 2, CD 16, CD 38, and CD 56, but not CD 3, CD 4, and CD 8, so they were natural killer(NK) cells. Splenectomy was performed and the operative specimen showed diffuse infiltration of pleomorphic lymphocytes, probably chronic lymphocytic leukemia cells. After splenectomy, the platelet count returned to normal but the lymphocytosis continued. Two years after discharge, chemotherapy was done because of thrombocytopenia and hepatomegaly. The patient died of disseminated intravascular coagulation arising from sepsis. The differences and similarities between peripheral and lymph-node lymphocytes suggest that LGL and NK cells may be differentiated from the same kind of cell, somewhat differentiated from stem cells.
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