Granular lymphocytic leukaemia (GLL)

Last updated: Friday, 03, December, 2010
SubtypesAppropriate Tests

T-GLL

Associations with pure red cell aplasia and rheumatoid arthritis are recognised.

A rare lymphoproliferative disorder characterised by increased numbers of large granular lymphocytes of clonal T cell (T-GLL) or natural killer (NK ) cell origin (NK-GLL).

Other causes of granular lymphocytosis include reactive states.

See also:

Splenomegaly, Neutropaenia, Lymphocytosis and cell immunophenotyping.

FBP and film: neutropaenia is a common association.

Flow cytometry / immunopenotyping of lymphocytes: The pathological cell population expresses CD3 with the common variant co-expressing CD8 and CD57. Other rare variants are recognised that co-express CD4 or both CD4 and CD8. CD56 may also be expressed. Aberrant loss of other pan T-cell markers and/or over expression of CD16 may be seen.

T-cell receptor (TCR) gene rearrangement studies: May confirm clonality.

Aggressive NK-Cell leukaemia:

This condition commonly presents with fever, hepatosplenomegaly and lymphadenopathy with a leukaemia blood film.

FBP and film: anaemia, neutropaenia and thrombocytopaenia are common.

Flow cytometry / immunopenotyping of lymphocytes: The pathological cell population expresses CD2 and CD56 in the absence of CD3. CD11b and CD16 may be expressed. CD57 is often negative.

Assessment of KIR antigen status may assist in establishing clonality.

TCR gene rearrangement studies: TCR genes are not rearranged.

Indolent NK-cell lymphoproliferative disorder: aka chronic NK-cell lymphocytosis / NK -cell large granular lymphocytosis.

Cases are typically asymptomatic, though co-existing vasculitis or nephrotic syndrome may rarely be seen.

FBP and film: anaemia, neutropaenia and thrombocytopaenia are common.

Flow cytometry / immunopenotyping of lymphocytes: The pathological cell population expresses CD2, CD16 and CD56 and CD57 in the absence of CD3.

TCR gene rearrangement studies: TCR genes are not rearranged.

References:

Jaffe et al. World Health Organisation Classification of Tumors - Pathology and Genetics of Tumors of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon, 2001.