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Survivin expression in the bone marrow of patients with severe congenital neutropenia

Leukemia volume 23, pages 622–625 (2009)Cite this article

Survivin, an inhibitor of apoptosis protein (IAP) family member, is expressed during fetal development and was reported to be undetectable in terminally differentiated adult tissues.1 More recent studies have shown that survivin expression is also high in immature bone marrow neutrophil precursors and can be detected in terminally differentiated neutrophils from patients with chronic inflammation.2 Survivin is also prominently expressed in most common cancers and is associated with chemoresistance and poor prognosis, making survivin-targeted therapies an attractive cancer treatment strategy.1, 3

Severe congenital neutropenia (SCN) is a heterogenous group of primary immunodeficiencies in which a lack of neutrophils causes inadequate immune responses to bacterial infections. Patients suffering from autosomal recessive SCN (also known as Kostmann disease) display a characteristic maturation arrest at the promyelocyte/myelocyte stage of myelopoiesis, and our previous studies have demonstrated that this maturation arrest is associated with mitochondria-dependent apoptosis of myeloid progenitor cells.4 Moreover, recent studies have identified homozygous mutations in the antiapoptotic gene encoding HAX-1 in patients with Kostmann disease, including patients belonging to the original kindred described by Rolf Kostmann in northern Sweden.5

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Acknowledgements

This study was supported by the Swedish Cancer Foundation, Swedish Children's Cancer Foundation, Lars Hierta Memorial Foundation and Stockholm County Council (ALF Project).

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Authors and Affiliations

  1. Department of Woman and Child Health, Childhood Cancer Research Unit, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden

    G Carlsson, S Boxhammer & J I Henter

  2. Division of Biochemical Toxicology, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden

    S Boxhammer & B Fadeel

  3. Division of Clinical Chemistry and Pharmacology, Uppsala University Hospital, Uppsala, Sweden

    D Garwicz

  4. Department of Medicine, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden

    J Palmblad

  5. Department of Molecular Medicine, Clinical Genetics Unit, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden

    M Nordenskjöld

  6. Department of Pathology, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden

    A Porwit

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  1. G Carlsson
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  2. S Boxhammer
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  3. D Garwicz
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  7. A Porwit
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  8. B Fadeel
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Correspondence to B Fadeel.

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Carlsson, G., Boxhammer, S., Garwicz, D. et al. Survivin expression in the bone marrow of patients with severe congenital neutropenia. Leukemia 23, 622–625 (2009). https://doi.org/10.1038/leu.2008.258

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