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Clonal Bone Marrow Failure Overlap Syndromes

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Chronic Myeloid Neoplasias and Clonal Overlap Syndromes

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Abstract

Among acquired stem cell disorders aethiopathological links have been established between hypoplastic MDS, aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH) and T-cell large granular lymphocytic leukemia (T-LGL) (see Fig. 10.1). All these entities are bone marrow failure disorders1 in which oligoclonal T-cell-mediated immune responses are without doubt pathophysiologically relevant. These overlap syndromes seem to form some kind of disease-continuum, whereby each entity can occur on its own, or arise in the background of any of the other above-mentioned diseases. As an example, PNH may follow, or precede MDS, and MDS-clones as well as PNH-clones are often detectable in patients with aplastic anemia. It may well be that T-LGL represents one extreme end of this spectrum, characterized by maximal clonal/oligoclonal T-cell proliferation, as LGL-like immunodominant cytotoxic lymphocyte (CTL) clonotypes are found within the whole spectrum of this continuum of overlap syndromes [2].

Overview of overlap syndromes

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  1. Universitätsklinik für Innere Medizin III, Paracelsus Medizinische Privatuniversität, Salzburg, Austria

    Dr. med. Dipl. Ing. biomed. inf. Lisa Pleyer (Editor), PD Dr. med. Daniel Neureiter M.A. (Editor) & Univ.-Prof. Dr. med. Richard Greil (Editor)

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  1. Dr. med. Dipl. Ing. biomed. inf. Lisa Pleyer
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  2. PD Dr. med. Daniel Neureiter M.A.
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  3. Univ.-Prof. Dr. med. Richard Greil
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  1. Universitätsklinik für Innere Medizin III, Paracelsus Medizinische Privatuniversität, Salzburg, Austria

    Richard Greil , Lisa Pleyer  & Viktoria Faber ,  & 

  2. Universitätsinstitut für Pathologie, Paracelsus Medizinische Privatuniversität, Salzburg, Austria

    Daniel Neureiter

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Pleyer, L., Neureiter, D., Greil, R. (2010). Clonal Bone Marrow Failure Overlap Syndromes. In: Greil, R., Pleyer, L., Faber, V., Neureiter, D. (eds) Chronic Myeloid Neoplasias and Clonal Overlap Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-79892-8_10

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