Von Willebrand factor, angiotensin converting enzyme and endothelial cell damage in veno-occlusive disease and bone marrow transplantation

1992 ◽  
Vol 66 (5) ◽  
pp. 617-618 ◽  
Author(s):  
Andrew Blann ◽  
Peter Collins
1995 ◽  
Vol 89 (3) ◽  
pp. 261-265 ◽  
Author(s):  
Shang-Der Shieh ◽  
Jeng-Chuan Shiang ◽  
Yuh-Feng Lin ◽  
Wen-Yen Shiao ◽  
Jia-Yi Wang

1. Military recruits frequently succumb to exertional heat stroke during intensive training. Since widespread endothelial injury is often associated with exertional heat stroke, the relationship between changes in three circulating endothelial cell markers (angiotensin-converting enzyme, von Willebrand factor antigen and thrombomodulin) and exertional heat stroke was studied. 2. Twelve recruits who had succumbed to exertional heat stroke during basic physical training (5000 m running) were included in the study. Another 10 age-matched healthy subjects who had gone through the same physical training regimen were selected as controls. 3. Blood was withdrawn on admission and on discharge for analyses of angiotensin-converting enzyme, von Willebrand factor antigen and thrombomodulin. Other physiological parameters and biochemical analyses reflecting renal and liver functions were also recorded. 4. Our results indicated that these subjects with exertional heat stroke exhibited impaired liver function as revealed by the significant elevation of both serum glutamic oxaloacetic transaminase (P < 0.05) and serum glutamic pyruvic transaminase (P < 0.05) as compared with normal healthy control subjects. Unfortunately, these values remained mostly somewhat elevated on discharge, although serum glutamic oxaloacetic transaminase was reduced dramatically. Indices of kidney functions, including creatinine clearance and uric acid and phosphorus secretion, were not significantly different from those observed in healthy controls. 5. Circulating angiotensin-converting enzyme activities in exertional heat stroke patients on admission were significantly lower than in normal subjects (10.68 ± 2.15 versus 21.21 ± 3.18 nmol hippuric acid min−1ml−1, P<0.05). In contrast, von Willebrand factor antigen, thrombomodulin and plasma renin activity were significantly elevated compared with corresponding values in healthy subjects (P < 0.001, 0.05 and 0.01 respectively). The values of these endothelial markers returned to normal on discharge. 6. These data suggest that the decrease in angiotensin-converting enzyme and increase in von Willebrand factor antigen and thrombomodulin associated with exertional heat stroke may reflect endothelial injuries. Since endothelial injuries may trigger haemostatic failure and/or disseminated intravascular coagulation, the monitoring of these values might be useful for the evaluation of endothelial status in patients suffering from exertional heat stroke.


Blood ◽  
1999 ◽  
Vol 93 (11) ◽  
pp. 3798-3802 ◽  
Author(s):  
R. Martijn van der Plas ◽  
Marion E. Schiphorst ◽  
Eric G. Huizinga ◽  
Ronald J. Hené ◽  
Leo F. Verdonck ◽  
...  

Abstract Thrombotic thrombocytopenic purpura (TTP) after bone marrow transplantation (BMT) differs from classic TTP in its clinical course and therapy. A characteristic of classic TTP is the inhibition of a plasma protease that specifically cleaves von Willebrand factor (vWF), thus reducing its multimeric size. We investigated whether this protease was also inhibited in BMT-associated TTP. Plasma from patients with classic or BMT-associated TTP was incubated with recombinant vWF R834Q, a vWF mutant with enhanced sensitivity to the protease. The proteolysis of vWF multimers was analyzed and quantified on Western blot. Metalloprotease activity was strongly inhibited in the classic TTP patient group. However, metalloprotease activity was normal in the BMT-associated TTP patient group. The difference in activity between the two patient groups was highly significant (P = .0016). The results indicate that the etiologies of classic and BMT-associated TTP are indeed different and provide an explanation for the lack of success of plasma exchange in BMT-associated TTP.


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