Demolombe-Ragué S, Pinède L, Ninet J, Bouchou K, Duhaut P, Berger F, Boucheron S, Rousset H, Pasquier J
Pavillon H, Service de Médecine interne, Hôpital Edouard Herriot, Lyon.
Presse Med. 1995 Nov 25;24(36):1689-93.
Malignant angioendotheliomatosis is an intravascular proliferation of tumour cells in the small arteries, veins and capillaries. Its lymphomatous origin has been proved recently by immunohistochemistry. It is a rare, generally rapidly fatal condition. Less than 150 cases have been reported in the literature. We report three cases. Long course fever with delayed neurologic disturbances were observed in the first case and long course fever, hyponatraemia, corticotropic and gonadotropic insufficiency in the second. The third case began with febrile meningo-encephalitis. Diagnosis was made at autopsy in the two first cases and on evidence from kidney needle-biopsy in the third. Clinical manifestations are polymorphic and frequently simulate vasculitis. Neurologic signs are the most common. Histologically, kidneys, skin, endocrine glands, lungs, muscles and most of the organs may be involved. Surprisingly lymph nodes, bone marrow and spleen are often free from disease. Current treatment is based on polychemotherapy. Earlier diagnosis of the disease should improve the prognosis as observed in our third patient.
恶性血管内皮瘤病是一种肿瘤细胞在小动脉、静脉和毛细血管内的增殖性疾病。其淋巴瘤起源最近已通过免疫组织化学得到证实。这是一种罕见的、通常迅速致命的疾病。文献报道不足150例。我们报告3例。第1例观察到长期发热伴延迟性神经功能障碍，第2例观察到长期发热、低钠血症、促肾上腺皮质激素和促性腺激素功能不全。第3例以发热性脑膜脑炎起病。前2例在尸检时确诊，第3例根据肾穿刺活检结果确诊。临床表现多样，常酷似血管炎。神经体征最为常见。组织学上，肾脏、皮肤、内分泌腺、肺、肌肉和大多数器官均可受累。令人惊讶的是，淋巴结、骨髓和脾脏通常无病变。目前的治疗基于多药化疗。如我们的第3例患者所示，早期诊断该病应可改善预后。
