Perronne Véronique, Roberts-Harewood Marilyn, Bachir Dora, Roudot-Thoraval Françoise, Delord Jean-Marie, Thuret Isabelle, Schaeffer Annette, Davies Sally C, Galactéros Frédéric, Godeau Bertrand
Centre de la drépanocytose, Hôpital Henri Mondor, Créteil, France.
Hematol J. 2002;3(1):56-60. doi: 10.1038/sj.thj.6200147.
An understanding of the causes of death among patients with sickle cell disease may be informative for both epidemiology and pathogenesis. This information should aid anticipation of dangerous clinical conditions, counselling patients and design of preventive therapies.
All deaths known to four European sickle cell disease centres over a 10-year period were retrospectively analysed. The circumstances of death were classified as follows: (1) acute sickle related vaso-occlusion; (2) chronic organ failure related to sickle cell disease; (3) infection; and (4) miscellaneous causes.
Sixty-one adult patients (mean age: 32+/-11 years) died during the study period. Twelve patients suddenly died at home; most of them exhibited symptoms of vaso-occlusion but in eight patients, the cause of death was unknown. The primary cause of death in the 53 evaluable patients was sickle related vaso-occlusion (27 out of 53; 51%) which manifested mainly by acute multiorgan failure (n=13) and acute chest syndrome (n=9). Ten of the 27 patients (37%) who died in these circumstances had an apparent mild disease before their deaths. Ten patients (19%) died of documented infection. Ten of the evaluable patients (19%) died of a chronic terminal visceral involvement related to sickle cell disease which was mainly liver cirrhosis. Four patients died by suicide or because of refusal of care and two patients died of iatrogenic complication.
The primary cause of death in adults appears to be vaso-occlusive, even in patients with no overt organ-system failure. Our results emphasise that the circumstances of death in sickle cell disease are different between adults and children. The deaths among adults appear not to be easily assigned to a few preventable causes as they are in children.
了解镰状细胞病患者的死因对于流行病学和发病机制研究可能都具有参考价值。这些信息应有助于预测危险的临床状况、为患者提供咨询以及设计预防性治疗方案。
对欧洲四个镰状细胞病中心在10年期间已知的所有死亡病例进行回顾性分析。死亡情况分类如下：（1）急性镰状细胞相关血管阻塞；（2）与镰状细胞病相关的慢性器官衰竭；（3）感染；（4）其他原因。
在研究期间，61名成年患者（平均年龄：32±11岁）死亡。12名患者在家中突然死亡；其中大多数有血管阻塞症状，但8名患者死因不明。53例可评估患者的主要死因是镰状细胞相关血管阻塞（53例中的27例；51%），主要表现为急性多器官衰竭（n = 13）和急性胸部综合征（n = 9）。在这些情况下死亡的27例患者中有10例（37%）在死亡前病情明显较轻。10例患者（19%）死于确诊的感染。10例可评估患者（19%）死于与镰状细胞病相关的慢性终末期内脏受累，主要是肝硬化。4例患者死于自杀或拒绝治疗，2例患者死于医源性并发症。
即使在没有明显器官系统衰竭的患者中，成年人的主要死因似乎也是血管阻塞。我们的结果强调，镰状细胞病患者的死亡情况在成人和儿童之间有所不同。与儿童不同，成人的死亡似乎不容易归因于少数可预防的原因。
