Large granular lymphocytic leukemia
|Large granular lymphocytic leukemia|
|Classification and external resources|
It is sometimes known as T-cell large granular lymphocyte leukemia (specifying involvement of the T cells). However, NK large granular lymphocyte leukemia (involving natural killer cells) is also possible.
It is also known by the following terms: proliferation of large granular lymphocytes (LGLs), LGL leukemia, Tγ-lymphoproliferative disorder, and, in common with other T cell leukemias such as T-cell prolymphocytic leukemia, T-cell chronic lymphocytic leukemia.
The postulated cells of origin are a transformed CD8+ T-cell with clonal rearrangements of β chain T-cell receptor genes for the majority of cases and a CD8- T-cell with clonal rearrangements of γ chain T-cell receptor genes for a minority of cases.
This disease is known for an indolent clinical course and incidental discovery. The most common physical finding is moderate splenomegaly. B symptoms are seen in a third of cases, and recurrent infections due to the associated neutropenia are seen in almost half of cases.
Rheumatoid arthritis is commonly observed in patients with T-LGL, leading to a clinical presentation similar to Felty's syndrome. Signs and symptoms of anemia are commonly found, due to the association between T-LGL and erythroid hypoplasia.
Sites of involvement
The 5 year survival has been noted as 89% in at least one study from France of 201 patients with T-LGL leukemia.
The neoplastic cells of this disease display a mature T-cell immunophenotype, with the majority of cases showing a CD4-/CD8+ T-cell subset immunophenotype versus other permutations of those markers. Variable expression of CD11b, CD56, and CD57 are observed. Immunohistochemistry for perforin, TIA-1, and granzyme B are usually positive.
|Common type (80% of cases)||CD3+, TCRαβ+, CD4-, CD8+|
|Rare variants||CD3+, TCRαβ+, CD4+, CD8-|
|CD3+, TCRαβ+, CD4+, CD8+|
|CD3+, TCRγδ+, CD4 and CD8 variable|
Clonal rearrangements of the T-cell receptor (TCR) genes are a necessary condition for the diagnosis of this disease. The gene for the β chain of the TCR is found to be rearranged more often than the γ chain. of the TCR.
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