A PATIENT WITH WERNERS-SYNDROME AND ERYTHROLEUKEMIA - JUST COINCIDENCE

Citation
C. Bartal et al., A PATIENT WITH WERNERS-SYNDROME AND ERYTHROLEUKEMIA - JUST COINCIDENCE, Leukemia & lymphoma, 21(5-6), 1996, pp. 509-513
Citations number
48
Categorie Soggetti
Hematology
Journal title
ISSN journal
10428194
Volume
21
Issue
5-6
Year of publication
1996
Pages
509 - 513
Database
ISI
SICI code
1042-8194(1996)21:5-6<509:APWWAE>2.0.ZU;2-J
Abstract
Werner's syndrome is a rare clinical entity and approximately 150 case s have been reported in the medical literature. Werner's syndrome, inh erited by autosomal recessive transmission, is characterized primarily by a short stature, premature greying and balding, trophic ulceration of the legs, diabetes mellitus and hypogonadism. These features combi ne to present a picture of adult progeria. In this brief report we des cribe a 51-year-old Bedouin male with Werner's syndrome, diagnosed as erythroleukemia (AML-6), and presenting as acute pancytopenia. The pat ient died two months after diagnosis. This is a rare case of erythrole ukemia in a patient with Werner's syndrome. We survey current knowledg e of the cytogenetic pathogenesis of Werner's syndrome and erythroleuk emia, and attempt to explain the possible link between these two rare syndromes.