血栓性血小板減少性紫斑病の1剖検例—中枢神経系における血管病変の局在について

AN AUTOPSY CASE OF THROMBOTIC THROMBOCYTOPENIC PURPURA: TOPOGRAPHY OF THE VASCULAR LESION IN THE CENTRAL NERVOUS SYSTEM

　抄録　血栓性血小板減少性紫斑病（TTP）の血栓は，内臓器における検索で，主として細動脈—毛細血管移行部に生じることが知られている。しかしながら，中枢神経系に関する知見は未だ乏しいことから，今回TTPの1剖検例に関して血栓分布に注目して検索を行い，特に大脳および小脳皮質における血栓形成部位について血管構築上の観点から検討を加えた。その結果，血栓は，内臓器，中枢神経系を通じていわゆる門脈系には形成されにくいこと，大脳皮質では第III層あるいは第IV層に，小脳皮質ではプルキンエ細胞層に多数分布しており，そこでは径20〜50μmの細動脈分岐部に主として生じていることが明らかとなった。

We reported the pathological findings of an autopsy case of thrombotic thrombocytopenic pur-pura, with special reference to the topography of vascular lesion in the central nervous system. Amorphous eosinophilic, PAS-positive thrombi,

endothelial proliferation and aneurysmal dilatation of affected vessels were prominent features in the visceral organs such as the heart, kidneys, liver, pancreas, adrenals, thyroid and alimentary tracts. Most of these vascular lesions were restricted to the arteriolar-capillary junctions.

There were no thrombi in the lungs as report-ed previously. In addition, the thrombi were rare-ly seen in so-called portal vessels existed in the liver, pancreas and anterior pituitary gland.

In the central nervous system, there were many vascular lesions similar to those of visceral organs in the cerebral gray matter, brain stem and cere-bellar cortex. A few lesions were also seen in the white matter, subarachnoid space and choroid plex-us. Many foci of chromatolytic neurons as well as a few petechial hemorrhages and minute in-farcts were observed in the parenchyma. The char-acteristic of the vascular lesions in the cerebral and cerebellar cortex was the sites of thrombi. They were predominantly located at the bifurca-tion of arterioles, 20---50 Am in diameter, and were numerous in the third or fourth cortical layer of the cerebrum, and in the Purkinje cell layer of the cerebellum.

Although the etiology of TTP is still unknown, the characteristic topography of vascular lesions suggests primary endothelial involvement in this disease.