造血干细胞移植后中枢神经系统并发症的临床研究

目的 探讨造血干细胞移植(HSCT)后中枢神经系统(CNS)并发症的发生率影响因素及预后,提高CNS并发症的诊断和治疗水平,从而改善此类患者的生存.方法 研究对象为自2001年5月至2007年12月在北京市道培医院行HSCT的640例患者,对其中发生CNS并发症患者的临床特点进行回顾性分析和研究.结果 640例HSCT患者中共57例发生了CNS并发症,发生率为8.9%.非血缘、单倍型和间胞相合HSCT后CNS并发症的发生率分别为12.0%(10/83),13.5%(39/289)和3.4%(8/237)(P<0.001).预处理为全身照射(TBI)和非TBI方案的发生率分别为19.4%(7/36)和8.3%(50/604)(P=0.047).年龄<14岁组和年龄≥14岁组的发生率分别为15.3%(9/59)和8.3%(48/581)(P=0.072).恶性疾病中的发病率为8.9%(56/627),非恶性疾病中的发病率为7.7%(1/13)(P=1.000).最常见的并发症为原发病复发和颅内感染.患者总体病死率为57.9%(33/57),其中66.7%(22/33)的患者死亡原因为CNS并发症.结论 单倍型和非血缘移植、TBI的预处理方案是移植后发生CNS并发症的高危因素.而年龄和原发病类型对CNS并发症的发病率无显著影响.早期诊断和积极有效地治疗CNS并发症可以降低其相关病死率,改善患者的预后.

A study of the central nervous system complications after hematopoietic stem cell transplantation

Objective To study the incidence, risk factors and prognosis of central nervous system (CNS) complications after hematopoietic stem cell transplantation ( HSCT) in order to prevent or reduce its occurrence, provide better diagnosis and treatment and improve the survival of the patients.Methods A total of 640 patients who consecutively underwent HSCT in our hospital between May 2001 and December 2007 were included.The clinical outcomes of the patients who developed CNS complications were analyzed.Results The patients received stem cells from haploidentical family members ( Haplo, n = 289 ) , identical siblings (IS, n = 237) , unrelated donors ( URD, n = 83) , unrelated cord blood (n = 14) , syngeneic siblings (n = 9 ) or autologous peripheral blood ( n = 8 ).Fifty-seven of 640 patients (8.9% ) developed CNS complications.The incidences were 12.0%, 13.5% and 3.4% in URD-HSCT, Haplo-HSCT and IS-HSCT respectively ( P <0.001).The incidences of CNS complications were 19.4% and 8.3% in cases who received or did not receive conditioning with TBI ( P = 0.047 ).There was no significant difference in the incidences of CNS complications between children (15.3% ) and adults(8.3% ) (P = 0.072).Similar incidences of CNS complications were seen in patients with hematological malignancies (8.9%) and non-malignant hematological disorders (7.7%)(P = 1.000).Five of the 57 patients developed two kinds of CNS complications.The patterns of CNS complications included relapse (17 cases) , infections (15 cases) , cyclosporine or FK506 encephalopathy (9 cases) , cerebral hemorrhage ( 8 cases) , cerebral infarction (2 cases), Wernicke's encephalopathy (1 case), skull fracture (1 case), drug-related meningitis (1 case), hepatic encephalopathy (3 cases), post-transplant lymphoproliferative disorder (1 case) and undetermined causes (4 cases).The overall mortality in the patients who developed CNS complications was 57.9% and 66.7% of them died of CNS complications.Conclusions CNS complications are not uncommon after HSCT and they have high mortality and poor prognosis.Our data suggest that haplo-HSCT,URD-HSCT and conditioning with TBI, but not the age and types of hematological diseases are the risk factors for development of CNS complications.Relapse and infections are the most common CNS complications in HSCT recipients.Early diagnosis and appropriate management are crucial to the improvement of clinical outcomes in these patients.