Association of vascular amyloid beta and cells of the mononuclear phagocyte system in hereditary cerebral hemorrhage with amyloidosis (Dutch) and Alzheimer disease.

[hereditary cerebral hemorrhage with amyloidosis]

Arterial and arteriolar amyloid-beta (A beta ) deposition in hereditary cerebral hemorrhage with amyloidosis (Dutch ) (HCHWA-D ) and Alzheimer disease (AD ) cerebral amyloid angiopathy (CAA ) were studied as to morphology , extent , and association with mononuclear phagocyte system (MPS ) cells using A beta , a-smooth muscle actin , and monocyte /macrophage marker (HLA-DR , CD 68 , CD 11 c , CD 4 5 ) immunohistochemistry . The HCHWA-D /AD arterial /arteriolar media showed compact A beta deposits , first appearing at the media /adventitia junction , and concomitant smooth muscle loss . Only HCHWA-D CAA featured (a ) severe involvement of larger arteries and (b ) arterioles showing a single or double ring of radial A beta surrounding compact A beta . Radial A beta appeared to develop at the media /adventitia junction . Monocyte /macrophage marker-positive foci /cells co -localized with HCHWA-D arterial A beta . Focal HLA-DR /CD 11 c positivity was observed at the media /adventitia junction of AD /HCHWA-D arteries in the absence of local A beta , but not in controls . Monocyte /macrophage marker positivity co -localizing with radial A beta appeared continuous with perivascular cells and microglia clustering perivascularly . These results suggest that (a ) MPS cells are topographically associated with HCHWA-D arterial A beta and radial arteriolar A beta , and (b ) HLA-DR /CD 11 c immunoreactivity may appear at the media /adventitia junction prior to A beta . The latter finding and the assumed formation of radial A beta at the media /adventitia junction may relate to involvement of the abluminal basement membrane in CAA pathogenesis . The role of MPS cells in this process remains to be established .

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hereditary cerebral hemorrhage with amyloidosis

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