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[Large granular lymphocytic leukemia]
S Berentsen1, O Bruserud, E Ulvestad
1Medisinsk avdeling, Fylkessjukehuset i Haugesund.
Insights
Large granular lymphocyte leukaemia (LGL leukaemia) is a rare T-cell chronic lymphocytic leukaemia. Diagnosis involves identifying CD3+, CD8+, CD57+ lymphocytes in patients with neutropenia and rheumatic symptoms.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Large granular lymphocyte leukaemia (LGL leukaemia) is a rare, chronic lymphoproliferative bone marrow disorder.
- It is considered a subtype of chronic T-cell lymphocytic leukaemia (T-CLL).
Observation:
- Three patients presented with chronic disease, neutropenia, and relative lymphocytosis.
- All patients exhibited an expansion of mature lymphocytes with a CD3+, CD8+, CD57+ immunophenotype.
- Two patients had recurrent infections, and all three showed serological findings compatible with rheumatic disease, including rheumatoid arthritis in two.
Findings:
- LGL leukaemia is characterized by lymphocytosis, neutropenia, and an increased susceptibility to infection.
- Associated autoimmune manifestations, such as rheumatoid arthritis, are frequently observed.
Implications:
- LGL leukaemia should be considered in patients with chronic neutropenia and lymphocytosis, especially with concurrent rheumatic symptoms.
- Flow cytometric immunophenotyping is a sensitive diagnostic tool for LGL leukaemia.
Abstract:
Large granular lymphocyte leukaemia (LGL leukaemia) is a rare, chronic lymphoproliferative bone marrow disease which can be considered a subtype of chronic T-cell lymphocytic leukaemia (T-CLL). We describe three patients with large granular lymphocyte leukaemia. They all suffered from chronic disease with neutropenia and relative lymphocytosis. An expansion of mature lymphocytes with the CD3+, CD8+, CD57+ immunophenotype was demonstrated in all three patients. Two patients had a history of recurring infections. Serological findings compatible with rheumatic disease were present in all three patients, and two suffered from rheumatoid arthritis. We have made a brief survey of this disease, which is characterized by relative or absolute lymphocytosis, neutropenia, and an increased risk of infection. Rheumatoid arthritis and other associated autoimmune manifestations occur frequently. Large granular lymphocyte leukaemia should be considered a possible diagnosis in patients with chronic neutropenia and relative lymphocytosis, in particular if rheumatic manifestations are also present. Flow cytometric immunophenotyping is a sensitive method in diagnosing this disease.