Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
OEHLER, L; REITER, E; FRIEDL, J; KABRNA, E; HAAS, OA; ROSENKRANZ, A; LECHNER, K; GEISSLER, K.
Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report.
Ann Hematol. 1994; 69(6):325-327
Doi: 10.1007/BF01696564
(- Case Report)
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Rosenkranz Alexander
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- Abstract:
- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Granulocyte Colony-Stimulating Factor - therapeutic use
- Hematopoiesis - drug effects
- Humans -
- Leukoplakia - complications
- Male -
- Nails, Malformed -
- Neutropenia - drug therapy
- Pigmentation Disorders - drug therapy
- Syndrome -
- X Chromosome -
- Find related publications in this database (Keywords)
- DYSKERATOSIS CONGENITA
- NEUTROPENIA
- G-CSF