Large granular lymphocyte leukaemia, T-LGL

Definition

There is a sustained increase in the number of large granular lymphocytes in the peripheral blood.

Synonyms

Epidemiology

Accounts for 2-3% of cases of small lymphocytic leukaemia 

Clinical features

60% of patients are symptomatic at presentation. Moderate splenomegaly1, rheumatoid arthritis1, circulating immune complexes and hypergammaglobulinaemia are common. Lymphadenopathy is uncommon1. Severe neutropenia is common1. Severe anaemia due to red cell hypoplasia may occur. The lymphocytosis usually exceeds 5.109/l.

Histopathology

 The large granular lymphocytes in blood and bone marrow have abundant cytoplasm with azurophilic granules. The bone marrow involvement is often interstitial, rarely nodular.

The spleen, which may weigh up to 1.8 kg, shows preservation of the architecture, with a lymphocytic infiltrate within the red pulp. Neoplastic cells infiltrate the walls of blood vessels. The white pulp is preserved, with the marginal zone demarcated by granzyme-negative lymphocytes. Germinal centres are present and may be prominent. In one cases, non-caseating granulomas were observed1.

Immunohistochemistry

 

CD2

positive

 

CD3

positive1

 

CD5

negative1

CD11b

variable

CD16

positive

CD45RO

negative1

CD56

variable

CD57

variable, more often in common type

TIA-1

usually positive, 5/51

Perforin

5/51

 

Granzyme B

3/41

 

bcl-2

negative1

 

MIB1

low expression1

 
 

The following variants exist:

 

common variant

rare variants

TCRab

positive

positive

positive

negative

TCRgd

negative

negative

negative

positive

CD4

negative1

positive

positive

not defined

CD8

positive1

negative

positive

not defined

Differential diagnosis

Management

Splenectomy is therapeutic in cases with hypersplenism1.

References

0 World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

1 Osuji N, Matutes E, Catovsky D, et al. Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. Am J Surg Pathol 2005; 29:935-41

This page last revised 24.9.2005.

©SMUHT/PW Bishop