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Treatment of the hematological manifestations of dyskeratosis congenita

Authors :
Putterman, C.
Safadi, R.
Zlotogora, J.
Banura, R.
Eldor, A.
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