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Introduction

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Large granular lymphocyte leukaemia (LGLL) may be of T lineage or natural killer (NK) lineage [1]. The former is dealt with in this section. It is mainly a disease of the elderly. There is no relationship to human lymphotropic viruses I and II [2].

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Clinical features

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Some diagnoses are incidental. Other patients present with symptoms resulting from cytopenia, e.g. infection as a result of neutropenia. Lymphadenopathy is quite uncommon. Splenomegaly is more common. Some patients have associated rheumatoid arthritis. Transformation to high-grade lymphoma is very rare [3].

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Haematological and pathological features

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There is an increase in large granular lymphocytes but the extent of this is very variable. The lymphocytes are very similar to normal large granular lymphocytes (Figure 18.1). In some patients there is neutropenia, anaemia with macrocytosis, anaemia with a low reticulocyte count (associated with red cell aplasia), anaemia with a high reticulocyte count (associated with autoimmune haemolytic anaemia) or thrombocytopenia. The bone marrow aspirate contains a variable number of large granular lymphocytes and in those with a complicating autoimmune cytopenia may show 'maturation arrest' in the granulocyte series, megaloblastic or macronormoblastic erythropoiesis, erythroid hyperplasia, pure red cell aplasia and increased or, less often, reduced megakaryocytes. Myeloid cells may be dysplastic. On trephine biopsy sections the usual pattern is of interstitial infiltration, which may be quite subtle (Figures 18.2 and 18.3). Infiltration within sinusoids and capillaries can also be a feature. There may be prominent lymphoid nodules composed of B cells and reactive T cells (Figures 18.2 and 18.4,18.5,18.6).

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Figure 18.1

Peripheral blood film from a patient with large granular lymphocyte leukaemia of T lineage showing two large granular lymphocytes. Romanowsky, x 100 objective.

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Figure 18.2

Bone marrow section in T-lineage large granular lymphocyte leukaemia showing a hypercellular bone marrow and an interstitial infiltrate with a reactive lymphoid nodule. H&E, x 20 objective.

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Figure 18.3

Bone marrow section in T-lineage large granular lymphocyte leukaemia showing an interstitial infiltrate by CD8-positive lymphocytes. Immunoperoxidase, x 20 objective.

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Figure 18.4

Bone marrow section in T-lineage large granular lymphocyte leukaemia showing CD20-positive lymphocytes in a reactive lymphoid nodule. Immunoperoxidase, x 10 objective.

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Figure 18.5

Bone marrow section in T-lineage large granular lymphocyte leukaemia showing CD4-positive lymphocytes in a reactive lymphoid nodule. Immunoperoxidase, x 20 objective.

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Figure 18.6

Bone marrow section in T-lineage large granular lymphocyte leukaemia showing CD3-positive ...

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