流行性 HUS
肾功能不全在大多数病例中是可逆的,在 2-3 周时间内缓解。大约 5% 的患者死于急性期,另外有 5% 可能有严重的后遗症(终末期肾脏疾病、大脑损伤)。[46]Siegler RL, Pavia AT, Christofferson RD. A 20-year population-based study of postdiarrheal hemolytic uremic syndrome in Utah. Pediatrics. 1994;94:35-40.http://www.ncbi.nlm.nih.gov/pubmed/8008534?tool=bestpractice.com[57]Garg AX, Suri RS, Barrowman N, et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome. JAMA. 2003;290:1360-1370.http://www.ncbi.nlm.nih.gov/pubmed/12966129?tool=bestpractice.com至少 25% 的患者将出现一定程度的慢性肾损害。[57]Garg AX, Suri RS, Barrowman N, et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome. JAMA. 2003;290:1360-1370.http://www.ncbi.nlm.nih.gov/pubmed/12966129?tool=bestpractice.com能够预测到不良结果的因素包括就诊时白细胞计数升高、无尿时间延长和老年人。[4]Banatvala N, Griffin PM, Greene KD, et al. The United States national prospective hemolytic uremic syndrome study: microbiologic, serologic, clinical and epidemiologic findings. J Infect Dis. 2001;183:1063-1070.http://www.ncbi.nlm.nih.gov/pubmed/11237831?tool=bestpractice.com[15]Pavia At, Nichols CR, Green DP, et al. Hemolytic-uremic syndrome during an outbreak of Escherichia coli O157:H7 infections in institutions for mentally retarded persons: clinical and epidemiologic observations. J Pediatr. 1990;116:544-551.http://www.ncbi.nlm.nih.gov/pubmed/2181098?tool=bestpractice.com[46]Siegler RL, Pavia AT, Christofferson RD. A 20-year population-based study of postdiarrheal hemolytic uremic syndrome in Utah. Pediatrics. 1994;94:35-40.http://www.ncbi.nlm.nih.gov/pubmed/8008534?tool=bestpractice.com[48]Dundas S Murphy J, Soutar RL, et al. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet. 1999;354:1327-1330.http://www.ncbi.nlm.nih.gov/pubmed/10533860?tool=bestpractice.com
散发性/继发性 HUS
因为具有这种诊断的患者在病例系列中一般是与血栓性血小板减少性紫癜 (TTP) 组合在一起,所以难以准确地估计预后。患有肺炎链球菌相关 HUS 的儿童住院期较长,所需透析次数较多,但是他们肾功能恢复的可能性较高。[58]Constantinescu AR, Bitzan M, Weiss LS, et al. Non-enteropathic hemolytic uremic syndrome: causes and short-term course. Am J Kidney Dis. 2004;43;976-982.http://www.ncbi.nlm.nih.gov/pubmed/15168377?tool=bestpractice.com继发于骨髓移植和化疗药物的 HUS 患者的预后不良。[59]Murgo A. Thrombotic microangiopathy in the cancer patient including those induced by chemotherapeutic agents. Semin Hematol. 1987;24:161-177.http://www.ncbi.nlm.nih.gov/pubmed/3310241?tool=bestpractice.com[60]Fuge R, Bird JM, Fraser A, et al. The clinical features, risk factors and outcome of thrombotic thrombocytopenic purpura occurring after bone marrow transplantation. Br J Haematol. 2001;113:58-64.http://www.ncbi.nlm.nih.gov/pubmed/11328282?tool=bestpractice.com[61]Kwaan HC, Gordon LI. Thrombotic microangiopathy in the cancer patient. Acta Haemat. 2001;106:52-56.http://www.ncbi.nlm.nih.gov/pubmed/11549777?tool=bestpractice.com尽管需要进一步的研究,但是与靶向抗癌药(例如免疫毒素类、单克隆抗体和酪氨酸激酶抑制剂)使用相关的血栓性微血管病患者报告表明,他们的结果要好于患有化疗相关血栓性微血管病的人们。[13]Blake-Haskins JA, Lechleider RJ, Kreitman RJ. Thrombotic microangiopathy with targeted cancer agents. Clin Cancer Res. 2011;17:5858-66.http://www.ncbi.nlm.nih.gov/pubmed/21813634?tool=bestpractice.com
家族性 HUS
进展为肾衰竭较为常见。[12]Warwicker P, Goodship TH, Donne RL, et al. Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int. 1998;53:836-844.http://www.ncbi.nlm.nih.gov/pubmed/9551389?tool=bestpractice.com[62]Ohali M, Shalev H, Schlesinger M, et al. Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H. Pediatr Nephrol. 1998;12:619-624.http://www.ncbi.nlm.nih.gov/pubmed/9811382?tool=bestpractice.com[63]Rougier N, Kazatchkine MD, Rougier JP, et al. Human complement factor H deficiency associated with hemolytic uremic syndrome. J Am Soc Nephrol. 1998;9:2318-2326.http://www.ncbi.nlm.nih.gov/pubmed/9848786?tool=bestpractice.com已有报告称血浆置换治疗可预防罕见病例中的肾衰竭。[41]Siegler RL. The hemolytic uremic syndrome. Pediatr Clin North Am. 1995;42:1505-1529.http://www.ncbi.nlm.nih.gov/pubmed/8614598?tool=bestpractice.com[64]Landau D. Shalev J, Levy-Finer G, et al. Familial hemolytic uremic syndrome associated with complement factor H deficiency. J Pediatr. 2001;138:412-417.http://www.ncbi.nlm.nih.gov/pubmed/11241053?tool=bestpractice.com依库珠单抗已显示出临床活性,但是未获得长期结果。[56]Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368:2169-2181.http://www.nejm.org/doi/full/10.1056/NEJMoa1208981#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/23738544?tool=bestpractice.com