Canterbury DHB

Context

Mature T- and NK-Cell Leukaemias

In This Section

T/NK Large Granular Lymphocyte Leukaemia

Mycosis/Fungoides/Sezary Syndrome

T/NK Large Granular Lymphocyte Leukaemia

This is a clonal disorder of cytotoxic T-lymphocytes. In the peripheral blood there is a persistent increase in Large Granular Lymphocytes (LGLs) of the order of >2 x 109/L. These cells are CD3, 8, 16 and 57 positive. There is a clonal T-cell receptor gene rearrangement.

Median age is about 60. Patients usually present with neutropenia and infections. Other cytopenias and splenomegaly may be seen. There is an association with rheumatoid arthritis.

Some patients pursue a very chronic course and do quite well with supportive care alone. Infections need to be treated promptly.

If treatment is given, low dose methotrexate is the initial treatment recommended. Cyclosporin, cyclophosphamide ± prednisone may also be effective.

Note: LGL leukaemia may also reflect an underlying indolent NK proliferation. In this situation, the abnormal LGLs are CD 2, 56, 57, 16 positive. They are CD 3 negative and the T-cell receptor genes are not rearranged. There is no association between this condition and cytopenias or rheumatoid arthritis.

For full discussion of LGL leukaemias, see:

Mycosis/Fungoides/Sezary Syndrome

Sezary Syndrome

This is a rare condition and is characterised by erythroderma, lymphadenopathy and neoplastic T-lymphocytes in the blood. It is traditionally regarded as a variant of Mycosis Fungoides, a T-Cell Lymphoma arising in the skin.

The circulating lymphoid cells may be small or large and are CD 2,3,4 and 5 positive. CD 7 is negative or weak and CD 8 is negative.

The disease is usually slowly progressive. Treatment when required is often unsatisfactory. Psoralen plus ultraviolet A (PUVA) may produce benefit.

About this Canterbury DHB document (5522):

Document Owner:

Andrew Butler (see Who's Who)

Issue Date:

September 2018

Next Review:

September 2021

Keywords:

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Topic Code: 5522