Mature T- and NK-Cell Leukaemias

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.

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:	Blake Hsu (see Who's Who)
Last Reviewed:	September 2018
Next Review:	September 2021
Keywords:
Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer The Haematology Department Protocols and Guidelines (the Red Book) are intended as a guide for registered health professionals to communicate guidelines and share best practices with supporting health professionals within New Zealand district health boards. They are not intended to be provided to or used as a reference for patients or non-registered health professionals. All health professionals must exercise their own clinical judgement and use pertinent clinical data when treating patients. The authors and editors of this publication have checked with sources believed to be reliable in their effort to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical science, neither the authors, editors, publisher, nor any other party who has been involved in preparing or publishing this work warrants that the information contained in it is accurate or complete in every respect. They therefore have no liability (whether in tort (including negligence) or otherwise) for any loss or damage arising out of any reliance on this information. This publication has been compiled by the staff and contractors of Canterbury District Health Board (CDHB) and as such CDHB has copyright for this book. Amendments must be authorised in accordance with the documented process. No release of part of, or all of, this publication to any other party or organisation is permissible without the prior approval of the designated document owner.
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