Wiskott-Aldrich syndrome.
Five cases of Wiskott-Aldrich Syndrome (WAS) were diagnosed in a tertiary care center in Saudi Arabia between 1981 and 1990. All five cases and two siblings (not reported here) had a history of rectal bleeding during the first week of life. Three of our patients were brothers. Two had classical Wiskott-Aldrich Syndrome and the third had the variant form of the syndrome. These two patients were successfully transplanted with allogenic bone marrow from their sisters. These two patients had complete engraftment with correction of both hematologic and immunologic abnormalities. Serum IgE returned to normal and their eczema cleared up. One patient died of intracranial hemorrhage. Two other patients had splenectomy. In these two splenectomized patients, platelet counts increased to normal values with no further bleeding. To our knowledge, this report is the first detailed description of WAS from Saudi Arabia.
Harfi
H A
HA
Division of Allergy and Clinical Immunology, Departments of Pediatrics and Medicine, and Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Al-Malik
S
S
Tulba
A
A