Abnormality of von Willebrand factor in patients with hemoglobin E-beta (0) thalassemia

American Journal of Clinical Pathology
P J BensonC M Smith

Abstract

The authors have identified six Southeast Asian patients ranging in age from 14 to 21 years with hemoglobin E-beta(0) thalassemia and a coagulopathy involving von Willebrand factor (vWF). These patients had normal or only slightly decreased plasma clotting factor levels. The activated partial thromboplastin time was prolonged in four of the patients. The abnormal feature common to all patients was a qualitative loss of high molecular weight multimers of vWF by crossed immunoelectrophoresis (vWF:CIE). Plasma vWF antigen concentration (vWF:Ag) and ristocetin cofactor activity (vWF:RCo) also were decreased and bleeding time prolonged in three patients. Epistaxis was present in two. No family history of increased bleeding tendency was present in any patient. Coagulation parameters and vWF:CIE were normal in two first-degree relatives without this hemoglobinopathy. vWF abnormalities and clinical manifestations were greatest in those patients with the most severe anemia and hepatosplenomegaly. These six patients appear to have an acquired abnormality of vWF, although they lack the clinical characteristics of acquired von Willebrand disease. While the etiology of this abnormality is unclear, the authors speculate that proteolysis of v...Continue Reading

Citations

Jul 11, 2006·American Journal of Hematology·Hiroshi Mohri
Mar 17, 2007·Critical Reviews in Clinical Laboratory Sciences·Massimo Franchini, Giuseppe Lippi
May 4, 2011·European Journal of Haematology·Shrimati ShettyKanjaksha Ghosh
Nov 30, 2006·American Journal of Hematology·Massimo Franchini, Giuseppe Lippi
Jun 1, 1996·Pediatric Clinics of North America·E J Werner

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