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T-Cell Lymphomas pp 137–153Cite as

The T-Cell Leukaemias

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Part of the book series: Contemporary Hematology ((CH))

Abstract

The mature T-cell leukaemias are a rare and heterogeneous group of disorders derived from the mature or post-thymic T-cell. They comprise T-cell prolymphocytic leukaemia, T-cell large granular lymphocytic leukaemia (T-LGL) and Adult T-cell lymphoma/leukaemia. The diagnosis of the T-cell leukaemias is based on a multiparameter approach which encompasses clinical presentation, peripheral blood count and morphology, immunohistochemistry, flow cytometry, cytogenetics and molecular genetics. Thus, the recent advances in modern immunophenotypic and molecular tools have been crucial in characterising these disorders and in distinguishing them from their B-cell counterparts. Prognosis and response to conventional chemotherapy are generally poor, with the exception of T-LGL which is a more indolent disorder than the others in its class. The rarity of these conditions, their refractoriness to standard therapies, underlying immune suppression, multi-factorial aetiologies and lack of single identifiable therapeutic targets in the majority of cases all contribute to a great management challenge. An important aspect therefore in advancing treatment of these and other T-cell disorders is adopting an international approach to diagnosis, documentation and trial design and entry in order to recruit sufficient patient and clinico-pathological data to inform robust choices.

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Authors and Affiliations

  1. Department of Haemato-Oncology, The Royal Marsden Hospital and Institute of Cancer Research, Downs Road, Sutton, Surrey, SM2 5PT, UK

    Elisabeth Grey-Davies

  2. Department of Haemato-Oncology, The Royal Marsden Hospital and Institute of Cancer Research, London, UK

    Claire Dearden

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  1. Elisabeth Grey-Davies
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  2. Claire Dearden
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Correspondence to Elisabeth Grey-Davies .

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Editors and Affiliations

  1. Yale University School of Medicine, Dept. of Medical Oncology/Hematology, Cedar Street 333, New Haven, 06520, Connecticut, USA

    Francine Foss

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Grey-Davies, E., Dearden, C. (2013). The T-Cell Leukaemias. In: Foss, F. (eds) T-Cell Lymphomas. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-170-7_9

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