X-linked thrombocytopenia and thrombocytopathia: attenuated Wiskott-Aldrich syndrome. Functional and morphological studies of platelets and lymphocytes

• Published in: Thrombosis and haemostasis Vol. 65; no. 3; p. 300
• Main Authors: Stormorken, H, Hellum, B, Egeland, T, Abrahamsen, T G, Hovig, T
• Format: Journal Article
• Language: English
• Published: Germany 04.03.1991
• Subjects: Adenosine Diphosphate - blood; Adenosine Triphosphate - blood; Adolescent; Bleeding Time; Blood Coagulation - physiology; Blood Platelet Disorders - blood; Blood Platelets - pathology; Blood Platelets - physiology; Genetic Linkage; Humans; Lymphocytes - pathology; Lymphocytes - physiology; Male; Pedigree; Platelet Count; Platelet Membrane Glycoproteins - metabolism; Severity of Illness Index; Thrombocytopenia - blood; Thrombocytopenia - genetics; Wiskott-Aldrich Syndrome - blood; Wiskott-Aldrich Syndrome - genetics; X Chromosome

Detailed studies on the rare disorder X-linked thrombocytopenia showed that it resembles the Wiskott-Aldrich syndrome (WAS) in inheritance, clinical bleeding tendency, platelet morphology, marked thrombocytopenia and microplatelets. The calculated platelet mass was 5% of normal. Functional and bioch...