Type I von Willebrand disease, subtype ‘platelet low’: decreased platelet adhesion can be explained by low synthesis of von Willebrand factor in endothelial cells
| Autor: | Jan J. Sixma, Marco Moia, Philip G. de Groot, Augusto B. Federici, Pier Mannuccio Mannucci, M. J. W. Ijsseldijk |
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| Rok vydání: | 1993 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities medicine.medical_specialty Umbilical Arteries Umbilical vein Extracellular matrix Platelet Adhesiveness Von Willebrand factor hemic and lymphatic diseases Internal medicine Platelet adhesiveness von Willebrand Factor medicine Von Willebrand disease Humans Platelet Cells Cultured biology Chemistry Infant Newborn Hematology Adhesion medicine.disease Endothelial stem cell von Willebrand Diseases Endocrinology cardiovascular system biology.protein Female Endothelium Vascular circulatory and respiratory physiology |
| Zdroj: | British Journal of Haematology. 83:88-93 |
| ISSN: | 1365-2141 0007-1048 |
| DOI: | 10.1111/j.1365-2141.1993.tb04636.x |
| Popis: | Endothelial cells (EC) were isolated from the umbilical vein of a newborn girl with type I 'platelet low' von Willebrand disease (I vWD) and endothelial localization and release and the ability of subendothelial von Willebrand factor (vWF) to support platelet adhesion were compared with those of normal EC. vWF was detectable by immunofluorescence in Weibel-Palade bodies, but the number of Weibel-Palade bodies positive for vWF was lower than in control EC. Patient EC released into the medium significantly smaller amount of vWF, both constitutively and after their stimulation. The vWF content of the extracellular matrix of patient EC was 38% that of control EC matrix. Platelet adhesion studies were performed under flow conditions with umbilical arteries and EC matrices of cultured EC. Using normal citrated whole blood as perfusate, platelet adhesion was lower in the umbilical artery of the patient (9 +/- 1% v 35 +/- 4% for the control) and in her EC matrix (7 +/- 1% v 21 +/- 2% of control). When patient EC matrix was perfused with vWF-deficient reconstituted blood, adhesion was 17 +/- 3% v 32 +/- 3% for control EC matrix; preincubation of patient EC matrix with 1 U/ml vWF increased the adhesion to 30 +/- 6%. These data establish that low contents of vWF in EC and subendothelium are important characteristics of type I vWD 'platelet low', and that such characteristics correlate with low platelet adhesion to the subendothelium. |
| Databáze: | OpenAIRE |
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