Department of Medicine, College of Medicine, College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK 73126-0901, USA.
Am J Hematol. 2011 Sep;86(9):743-51. doi: 10.1002/ajh.22091.
The absence of specific diagnostic criteria, the urgency to begin plasma exchange treatment, and the risk for complications from plasma exchange make the initial evaluation of patients with suspected thrombotic thrombocytopenic purpura (TTP) difficult. Systemic infections may mimic the presenting clinical features of TTP. In the Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, 1989-2010, 415 consecutive patients have been clinically diagnosed with their first episode of TTP; in 31 (7%) the presenting clinical features were subsequently attributed to a systemic infection. All 31 patients had diagnostic criteria for TTP; 16 (52%) had the complete "pentad" of microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal failure, and fever. Four (16%) of 25 patients who had ADAMTS13 measurements had <10% activity; three patients had a demonstrable ADAMTS13 inhibitor. Compared with 62 patients with severe ADAMTS13 deficiency (<10%) who had no recognized alternative disorders, patients with systemic infections had more frequent fever, coma, renal failure, and the complete "pentad" of clinical features. Seventeen different infectious etiologies were documented. A systematic literature review identified 67 additional patients with a diagnosis of TTP or HUS and also a systemic infection. Among all 98 patients, infections with 41 different bacteria, viruses, and fungi were documented, suggesting that many different systemic infections may mimic the presenting clinical features of TTP. Initial plasma exchange treatment is appropriate in critically ill patients with diagnostic features of TTP, even if a systemic infection is suspected. Continuing evaluation to document a systemic infection is essential to determine the appropriateness of continued plasma exchange.
缺乏特异性诊断标准、迫切需要开始血浆置换治疗以及血浆置换相关并发症风险使得疑似血栓性血小板减少性紫癜（TTP）患者的初始评估变得困难。全身感染可能会模仿 TTP 的临床表现。在俄克拉荷马州 TTP-HUS（溶血尿毒综合征）登记处，1989 年至 2010 年期间，连续有 415 例患者经临床诊断为首次发作 TTP；其中 31 例（7%）的临床表现随后归因于全身感染。所有 31 例患者均符合 TTP 的诊断标准；16 例（52%）具有微血管性溶血性贫血、血小板减少、神经功能异常、肾衰竭和发热的完整“五联征”。25 例接受 ADAMTS13 测量的患者中有 4 例（16%）<10%的活性；3 例患者存在可检测的 ADAMTS13 抑制剂。与 62 例无明确其他疾病的严重 ADAMTS13 缺乏症（<10%）患者相比，具有全身感染的患者更常出现发热、昏迷、肾衰竭和完整的“五联征”临床表现。记录了 17 种不同的感染病因。系统文献回顾确定了另外 67 例 TTP 或 HUS 诊断且伴有全身感染的患者。在所有 98 例患者中，记录了 41 种不同细菌、病毒和真菌的感染，表明许多不同的全身感染可能会模仿 TTP 的临床表现。对于具有 TTP 诊断特征且病情危急的患者，即使怀疑存在全身感染，初始血浆置换治疗也是合适的。继续评估以确定全身感染对于确定是否继续进行血浆置换至关重要。
