(11) Dyskeratosis congenita with pancytopenia
β Scribed by DRS H. BAKER; B. COLVIN; M.T. GLOVER
- Book ID
- 108661963
- Publisher
- John Wiley and Sons
- Year
- 1991
- Tongue
- English
- Weight
- 468 KB
- Volume
- 125
- Category
- Article
- ISSN
- 0007-0963
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## ABSTRACT Dyskeratosis congenita is a rare inheritable disorder characterized by abnormalities of the skin, nails and oral mucosa. Aplastic anaemia resulting from bone marrow hypoplasia is a frequent cause of death. Squamous cell carcinoma developing from leukoplakia and visceral malignancies are
Dyskeratosis congenita (DC) is a rare hereditary disorder of skin which may be associated with aplastic anemia. The pattern of inheritance is X-linked recessive in most instances, but autosomal dominant and autosomal recessive types have been documented. Reticulated hyperpigmentation usually is the