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Large granular lymphocytic leukemia

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Large granular lymphocytic leukemia

Large granular lymphocytic leukemia
Classification and external resources
ICD-O: 9831/3
MeSH D054066

Large granular lymphocytic leukemia is a disease that exhibits an unexplained, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood.[1]

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.[2]

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.[1]

Epidemiology

T-LGL is a rare form of leukemia, comprising 2-3% of all cases of small lymphocytic leukemias.[1]

Etiology

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.[1]

Clinical features

Clinical presentation

This disease is known for an indolent clinical course and incidental discovery.[1] 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.[3][4][5][6]

Rheumatoid arthritis is commonly observed in patients with T-LGL, leading to a clinical presentation similar to Felty's syndrome.[7] Signs and symptoms of anemia are commonly found, due to the association between T-LGL and erythroid hypoplasia.[8]

Laboratory findings

The requisite lymphocytosis of this disease is typically 2-20x109/L.[8]

Immunoglobulin derangements including hypergammaglobulinemia, autoantibodies, and circulating immune complexes are commonly seen.[6][9][10][11]

Sites of involvement

The leukemic cells of T-LGL can be found in peripheral blood, bone marrow, spleen, and liver. Nodal involvement is rare.[1][3]

Prognosis

The 5 year survival has been noted as 89% in at least one study from France of 201 patients with T-LGL leukemia.[12]

Morphology

Peripheral blood

The neoplastic lymphocytes seen in this disease are large in size with azurophilic granules that contains proteins involved in cell lysis such as perforin and granzyme B.[13]

Bone marrow

Bone marrow involvement in this disease is often present, but to a variable extent. The lymphocytic infiltrate is usually interstitial, but a nodular pattern rarely occurs.[1]

Molecular Findings

Immunophenotype

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.[4][5] Variable expression of CD11b, CD56, and CD57[6] are observed. Immunohistochemistry for perforin, TIA-1, and granzyme B are usually positive.[1]

Type Immunophenotype
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

Genetic findings

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.[10][14]

Treatment

Alemtuzumab has been investigated for use in treatment of refractory T-cell large granular lymphocytic leukemia.[15]

References

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