Hassell K L, Eckman J R, Lane P A
Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver.
Am J Med. 1994 Feb;96(2):155-62. doi: 10.1016/0002-9343(94)90136-8.
The purpose of this report is to characterize the acute multiorgan failure syndrome that complicates some episodes of sickle pain. A retrospective chart review was used to identify episodes of sickle pain complicated by the acute failure of at least two of three organs: lung, liver, or kidney. The defining criteria of organ failure were established, and the clinical characteristics, laboratory values, treatment methods, and outcomes were noted in episodes that met the criteria. Seventeen episodes of acute multiorgan failure were identified in 14 patients, 10 with sickle cell anemia and 4 with hemoglobin SC disease. Most episodes occurred during a pain event that was unusually severe for the patient. The onset of organ failure was associated with fever, rapid fall in hemoglobin level and platelet count, nonfocal encephalopathy, and rhabdomyolysis. Bacterial cultures were negative in all but four episodes. Aggressive transfusion therapy was associated with survival and with rapid recovery of organ function in all but one episode. The syndrome developed in patients who had previously exhibited relatively mild disease with little evidence of chronic organ damage and relatively high hemoglobin values in steady state. Acute multiorgan failure syndrome is a severe, life-threatening complication of pain episodes in patients with otherwise mild sickle cell disease. The syndrome appears to be reversed with prompt, aggressive transfusion therapy. High baseline hemoglobin levels may represent a predisposing factor.
本报告旨在描述并发于某些镰状细胞疼痛发作的急性多器官功能衰竭综合征。通过回顾性病历审查来确定并发至少三个器官（肺、肝或肾）中两个器官急性功能衰竭的镰状细胞疼痛发作。确立了器官功能衰竭的定义标准，并记录了符合标准发作的临床特征、实验室值、治疗方法及预后。在14例患者中识别出17次急性多器官功能衰竭发作，其中10例为镰状细胞贫血患者，4例为血红蛋白SC病患者。大多数发作发生在对患者而言异常严重的疼痛事件期间。器官功能衰竭的发作与发热、血红蛋白水平和血小板计数迅速下降、非局灶性脑病及横纹肌溶解有关。除4次发作外，所有细菌培养均为阴性。积极输血治疗除1次发作外均与生存及器官功能快速恢复有关。该综合征发生在先前表现为相对轻度疾病、几乎没有慢性器官损害证据且稳态血红蛋白值相对较高的患者中。急性多器官功能衰竭综合征是原本轻度镰状细胞病患者疼痛发作的一种严重、危及生命的并发症。该综合征似乎可通过及时、积极的输血治疗得到逆转。高基线血红蛋白水平可能是一个诱发因素。
