Horata-Takahashi E, Kamata K, Takeuchi Y, Kikawada R, Okubo M
Department of Internal Medicine, Kitasato University School of Medicine Kanagawa, Japan.
Nihon Jinzo Gakkai Shi. 1994 Jun;36(6):727-39.
Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) were originally described as separate disease entities. Recently, HUS and TTP have been considered a single disease, because of the identical microangiopathic lesion. In the present study, we investigated the clinical and histological characteristics of HUS/TTP. Eleven patients with a definite diagnosis of HUS/TTP were found from a cohort of adult patients who were admitted to Kitasato University Hospital in the past two decades. Their clinical and histological characteristics were retrospectively analyzed. All of the 11 patients with HUS/TTP were sporadic and non-diarrheal cases with a mean age of 49 years +/- 10. Preceding episodes of flu-like syndrome and the administration of mitomycin C were observed in 3 and 5 patients, respectively. On admission, two of 10 patients with renal dysfunction required dialysis treatment, while none developed nephrotic syndrome. Six patients showed CNS manifestation, such as consciousness disturbance and convulsion. Three patients with severe hypertension did not show consciousness disturbance. As for the final outcome, 6 patients recovered and the remaining 5 died. Two died after 60 hospital days. In the histopathological investigation, renal biopsy specimen showed narrowing of the capillary loops in the glomeruli due to swelling of the endothelial cells, double contour of the glomerular basement membrane, or mesangial cell necrosis and sclerosis. In the autopsy specimen, internal organ infarction with fibrin thrombi in small arteries was observed in multiple organs, such as brain, kidneys, hearts, lungs, jejunum, liver, pancreas, adrenal glands and pituitary gland. A circumferential myocardial infarction with hyaline thrombi in the medial layer of myocardium was characteristic of HUS/TTP. In conclusion, microangiopathic lesions with infarction spread widely throughout various organs in HUS/TTP. Involvement of internal organs, not to mention kidneys and brain, is lethal and their prognosis remains poor.
溶血性尿毒症综合征（HUS）和血栓性血小板减少性紫癜（TTP）最初被描述为不同的疾病实体。最近，由于微血管病变相同，HUS和TTP被视为单一疾病。在本研究中，我们调查了HUS/TTP的临床和组织学特征。在过去二十年入住北里大学医院的成年患者队列中，发现了11例确诊为HUS/TTP的患者。对他们的临床和组织学特征进行了回顾性分析。所有11例HUS/TTP患者均为散发性、非腹泻病例，平均年龄为49岁±10岁。分别在3例和5例患者中观察到流感样综合征前驱发作和丝裂霉素C的使用。入院时，10例肾功能不全患者中有2例需要透析治疗，而无一例发生肾病综合征。6例患者出现中枢神经系统表现，如意识障碍和惊厥。3例重度高血压患者未出现意识障碍。至于最终结局，6例患者康复，其余5例死亡。2例在住院60天后死亡。在组织病理学检查中，肾活检标本显示肾小球毛细血管袢因内皮细胞肿胀、肾小球基底膜双轮廓或系膜细胞坏死和硬化而变窄。在尸检标本中，在多个器官如脑、肾、心、肺、空肠、肝、胰、肾上腺和垂体中观察到小动脉内有纤维蛋白血栓的内脏梗死。心肌中层有透明血栓的环周性心肌梗死是HUS/TTP的特征。总之，HUS/TTP中伴有梗死的微血管病变广泛累及各个器官。内脏受累，更不用说肾脏和大脑，是致命的，其预后仍然很差。
