Summary
The immunocytochemical study of thrombomodulin (TM), a newly recognued anticoagulant
endothelial surface protein, was performed with a surgical specimen of a superficial
temporalartery (STA) obtained from a 29-year-old woman with familial moyamoya disease.
The staining of TM showed positive immunoreactivity in smaller vessels in the surrounding
connective tissue of the specimen, whereas negative in STA. Immunoelectronmicroscopically
the luminal plasma membrane of endothelial cells was positive for TM. These staining
pattern was the same as that in controls. She concurrently suffered from von Willebrand
disease type I, and she had two cerebral hemorrhagic attacks. A quantitative defect
of the von Willebrand factor in the endothelium was demonstrated immunocytochemically.
