ISSN:
1432-0584
Keywords:
Dyskeratosis congenita
;
Pancytopenia
;
Aplastic anemia
;
Granulocyte-macrophage colony
;
stimulating factor
;
Bone marrow transplantation
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01703237
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