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MDS as an Autoimmune Process

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The Myelodysplastic Syndromes

Abstract

The Myelodysplastic Syndromes (MDS) represent a markedly heterogeneous group of haematopoietic stem cell disorders, with a wide diversity in outcome. Whilst some cases of MDS carry an indolent course with prolonged survival, others may progress rapidly to acute myeloid leukaemia (AML) and death. The pathophysiology of MDS remains poorly understood, in part due to the heterogeneity of the disease. Several clonal karyotypic and genetic mutations are reported in MDS and likely to play a key role in its pathogenesis. Moreover, defects in immune responses, both innate and adaptive, together with, or in the absence of, altered cytokine/chemokine profiles are integral to the disease process. It is hypothesised that a combination of environmental factors, genetic background and autoimmune responses play role in the pathogenesis of MDS (Fig. 9.1). This chapter will focus on the immunological aspects of MDS and provide the clinical evidence to support MDS as an autoimmune disorder.

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Authors and Affiliations

  1. Department of Haematology, University Hospital of Wales, Heath Park, CF14 4XW, Cardiff, UK

    W. Ingram

  2. Department of Haematological Medicine, King’s College London, London, UK

    Y. Kordasti & G. J. Mufti

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  1. W. Ingram
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  2. Y. Kordasti
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  3. G. J. Mufti
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Correspondence to W. Ingram .

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  1. , 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, Budapest, 1125, Hungary

    Judit Várkonyi

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Ingram, W., Kordasti, Y., Mufti, G. (2011). MDS as an Autoimmune Process. In: Várkonyi, J. (eds) The Myelodysplastic Syndromes. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-0440-4_9

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