Kobayashi Y, Uoshima N, Hashiramoto K, Tanaka K, Kimura S, Wada K, Ozawa M, Maruo N, Kondo M, Minamikawa T
First Department of Internal Medicine, Kyoto Prefectural University of Medicine.
Rinsho Ketsueki. 1992 Sep;33(9):1279-84.
The patient was a 76-year-old female who had been referred to our hospital because of fever of unknown origin on October 15, 1987. On admission, the body temperature was 38.6 degrees C and atonic palsy of the left upper limb was noted. Abnormal laboratory findings included CRP5+, an increase in LDH, Hb 7.9 g/dl. The cause of the fever could not be identified. The fever did not respond to various treatment. The patient developed DIC in late October and died on November 5. In autopsy histological examination revealed tumor cells in the vessels of the generalized organs. A diagnosis of neoplastic angioendotheliosis (NAE) and immunohistologically B lymphoma was made. We reviewed the literature on 37 Japanese cases of NAE. The cases, consisting of 19 males and 18 females, were aged 37-87 years with a median value of 60 years. The symptoms observed during the course were most frequently mental or neurological symptoms and fever, and rash was uncommon. Laboratory findings were non-specific and biopsy was needed for definitive diagnosis. By autopsy, lesions were noted more frequently in the brain, kidneys, and lungs, and the findings in the skin were indeterminate. These observations suggest that when NAE should be considered, kidney, lung or skin biopsy should be performed for definitive diagnosis.
该患者为一名76岁女性，于1987年10月15日因不明原因发热被转诊至我院。入院时，体温为38.6摄氏度，左上肢出现弛缓性麻痹。实验室检查异常结果包括CRP5+、乳酸脱氢酶升高、血红蛋白7.9 g/dl。发热原因无法确定。各种治疗对发热均无反应。患者于10月下旬发生弥散性血管内凝血，并于11月5日死亡。尸检组织学检查发现全身各器官血管中有肿瘤细胞。诊断为肿瘤性血管内皮病（NAE），免疫组织化学诊断为B淋巴瘤。我们回顾了37例日本NAE病例的文献。这些病例包括19名男性和18名女性，年龄在37 - 87岁之间，中位数为60岁。病程中观察到的症状最常见的是精神或神经症状以及发热，皮疹并不常见。实验室检查结果不具有特异性，需要活检才能明确诊断。通过尸检发现，病变在脑、肾和肺中更为常见，皮肤病变情况不明确。这些观察结果表明，当考虑NAE时，应进行肾、肺或皮肤活检以明确诊断。
