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Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report

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Summary

Dyskeratosis congenita (DC) is a rare congenital X-linked disorder. The major clinical manifestations are abnormal skin pigmentation, nail dystrophy, and leukoplakia of mucosal membranes. About 50% of the patients develop bone marrow failure, which is partly responsible for the poor prognosis. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) has been administered to some neutropenic patients with DC, but only a moderate stimulation of neutropoiesis has been observed. We report on a patient with DC treated with recombinant human granulocyte-colony-stimulating factor (rhG-CSF). This treatment resulted in a substantial dose-dependent increase in the neutrophil count.

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Authors and Affiliations

  1. Department of Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Währinger Gürtel 18-20, A-1090, Vienna, Austria

    L. Oehler, E. Reiter, J. Friedl, E. Kabrna, K. Lechner & K. Geissler

  2. St. Anna Children's Hospital, Vienna, Austria

    O. A. Haas

  3. Department of Medicine III, Division of Nephrology, University of Vienna, Austria

    A. Rosenkranz

Authors
  1. L. Oehler
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  2. E. Reiter
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  3. J. Friedl
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  4. E. Kabrna
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  5. O. A. Haas
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  6. A. Rosenkranz
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  7. K. Lechner
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  8. K. Geissler
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Oehler, L., Reiter, E., Friedl, J. et al. Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report. Ann Hematol 69, 325–327 (1994). https://doi.org/10.1007/BF01696564

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  • DOI: https://doi.org/10.1007/BF01696564

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