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Treatment of the hematological manifestations of dyskeratosis congenita
- Source :
- Annals of Hematology; April 1993, Vol. 66 Issue: 4 p209-212, 4p
- Publication Year :
- 1993
-
Abstract
- Summary Dyskeratosis congenita is a congenital multisystem disorder, characterized by skin pigmentation, dystrophic nails, and leukoplakia. Hematologic abnormalities progressing to severe pancytopenia play a significant role in the poor prognosis of afflicted patients. We report on a patient with dyskeratosis congenita and severe aplastic anemia, complicated by life threatening infection. The patient was treated with recombinant granulocyte-macrophage colony-stimulating factor. This therapy resulted in a moderate and transient improvement in absolute neutrophil counts. Current concepts regarding the pathogenesis and etiology of dyskeratosis congenita are discussed, while reviewing the available therapeutic options.
Details
- Language :
- English
- ISSN :
- 09395555 and 14320584
- Volume :
- 66
- Issue :
- 4
- Database :
- Supplemental Index
- Journal :
- Annals of Hematology
- Publication Type :
- Periodical
- Accession number :
- ejs16163854
- Full Text :
- https://doi.org/10.1007/BF01703237