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Myelodysplasia and Naxos disease: a novel pathogenetic association?

Leukemia volume 16, pages 2335–2337 (2002)Cite this article

The myelodysplastic syndromes (MDS) are a heterogeneous group of blood neoplasms characterized by ineffective hemopoiesis and peripheral cytopenia, involving one or more of the non-lymphoid hemopoietic lineages and an increased tendency for evolution into acute leukemia. Although MDS are more often diagnosed in the elderly, clinically, morphologically and biologically similar diseases are also recognized in children. However, because of their lower incidence and clinical variability in the younger patient and the frequent overlap between the myelodysplastic and myeloproliferative syndromes, there is still considerable controversy on the most appropriate classification and optimal therapeutic approach for childhood MDS.

During childhood, MDS frequently arise from inherited constitutional genetic disorders regarded as a background predisposing to the acquisition of genomic lesions or the appearance of other forms of hemopoietic dysfunction. At least 30% of them affect patients with pre-existing inherited genetic disorders, mainly immunodeficiency states, constitutional chromosomal syndromes, DNA repair deficiency conditions and congenital cytopenias.1 Since the etiologic basis of myelodysplasia remains elusive, the study of such cases is expected to provide additional insight into the cascade of alterations leading to the dysplastic marrow failure.

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Acknowledgements

We thank Dr Peter Greenberg, Stanford University Medical Center, Stanford, CA for his helpful and critical comments.

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Authors and Affiliations

  1. Department of Pediatric Hematology/Oncology, ‘Aghia Sophia’ Children's Hospital, Athens, Greece

    S Polychronopoulou, JP Panagiotou & SA Haidas

  2. ‘Yannis Protonotarios’ Medical Center, Naxos, Greece

    A Tsatsopoulou & N Protonotarios

  3. Hematology Laboratory, ‘G Gennimatas’ General Hospital, Athens, Greece

    SI Papadhimitriou

  4. Department of Cardiology, Athens University Medical School, Athens, Greece

    A Anastasakis & N Protonotarios

  5. Laboratory of Immunology and Histocompatibility,‘G Gennimatas’ General Hospital, Athens, Greece

    G Paterakis

  6. Department of Hematopathology, ‘Evangelismos’ General Hospital, Athens, Greece

    D Anagnostou

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  1. S Polychronopoulou
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Polychronopoulou, S., Tsatsopoulou, A., Papadhimitriou, S. et al. Myelodysplasia and Naxos disease: a novel pathogenetic association?. Leukemia 16, 2335–2337 (2002). https://doi.org/10.1038/sj.leu.2402587

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  • DOI: https://doi.org/10.1038/sj.leu.2402587

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