 |
|
Fanconi-Like SyndromeImmunologic Deficiency, Pancytopenia, and Cutaneous Malignancies
David Abels, MD;
William B. Reed, MD
Arch Dermatol. 1973;107(3):419-423.
Abstract
A new familial heredodegenerative disorder with pancytopenia, immunologic deficiency, and cutaneous malignancies is reported. One patient had a prolonged course of multiple pneumothoraces, infections, and cutaneous malignancies. His immunologic abnormalities included near absence of IgA, elevated IgG with later development of abnormal  -globulin, and defective delayed hypersensitivity. At autopsy at age 23, no thymus gland was found. He also had secondary amyloidosis, possibly resulting from bilateral osteomyelitis of both legs due to Serratia which had become generalized at death. His younger brother had severe pancytopenia responsive to methyltestosterone therapy. The differential diagnosis of this disorder included Fanconi's anemia, dyskeratosis congenita, ataxia-telangiectasia, and the other similar known heredodegenerative disorders, but most likely represents a new disorder with similarities to all heredodegenerative disorders and to some other genetic immunologic disorders.
Author Affiliations
Santa Barbara, Calif; Irvine, Calif
From Santa Barbara General Hospital, Santa Barbara, Calif (Dr. Abels), and the University of California School of Medicine, Irvine (Dr. Reed).
Footnotes
Accepted for publication July 25, 1972.
Reprint requests to 1013 W Olive Ave, Burbank, Calif 91506 (Dr. Reed).
 CiteULike  Connotea  Delicious  Digg  Facebook  Reddit  Technorati  Twitter
What's this?
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Fanconi's Anemia: Tumor-like Warts, Hyperpigmentation Associated With Deranged Keratinocytes, and Depressed Cell-Mediated Immunity
Johansson et al.
Arch Dermatol 1982;118:249-252.
ABSTRACT
Epidermolysis Bullosa Dystrophica With Epidermal Neoplasms
Reed et al.
Arch Dermatol 1974;110:894-902.
ABSTRACT
|
