Fuge R, Bird J M, Fraser A, Hart D, Hunt L, Cornish J M, Goulden N, Oakhill A, Pamphilon D H, Steward C G, Marks D I
Bone Marrow Transplant Unit, Bristol Children's Hospital, United Bristol Healthcare Trust, Bristol, UK.
Br J Haematol. 2001 Apr;113(1):58-64. doi: 10.1046/j.1365-2141.2001.02699.x.
In this study, we retrospectively analysed the clinical features, risk factors and outcome of 22 patients with thrombotic thrombocytopenic purpura (TTP) occurring after allogeneic stem cell transplantation. All but two of these patients received stem cells from unrelated donors (UDs), two-thirds were female, three-quarters were adults and leukaemia was the major reason for transplant. The incidence of TTP was 20 out of 332 patients (6%) with UD transplants and two out of 104 recipients (2%) of matched sibling allografts (P = 0.16). In order to ascertain basic demographic risk factors for the development of TTP, we compared the 22 patients with 434 patients who did not develop TTP. Compared with patients who did not develop TTP, patients with TTP were nearly three times older (P < 0.001) and were more than twice as likely to be female (P = 0.001). Because > 90% of patients were recipients of UD marrow, we then compared the 20 UD-bone marrow transplantation (BMT) patients with 60 randomly selected UD-BMT patients who did not develop TTP. On univariate analysis, age and female gender were again significant risk factors, as was grade II-IV acute graft-versus-host disease (GvHD) (P = 0.002), and there was a trend towards an association with chronic GvHD (P = 0.083). However, after logistic regression analysis, only age and sex remained significant (P < 0.001 and 0.009 respectively). We report an 86% mortality with only three survivors out of 22 patients, and one of these remains thrombocytopenic and red cell transfusion dependent, possibly in part because of graft hypoplasia. Six out of 17 patients responded to plasmapheresis, but the majority of them ultimately succumbed because of TTP, often in association with GvHD or fungal infection.
在本研究中，我们回顾性分析了22例异基因干细胞移植后发生血栓性血小板减少性紫癜（TTP）患者的临床特征、危险因素及预后情况。这些患者中除2例以外均接受了非亲缘供者（UD）的干细胞，三分之二为女性，四分之三为成年人，白血病是移植的主要原因。TTP的发生率在332例接受UD移植的患者中为20例（6%），在104例接受同胞全相合移植的受者中为2例（2%）（P = 0.16）。为了确定发生TTP的基本人口统计学危险因素，我们将这22例患者与434例未发生TTP的患者进行了比较。与未发生TTP的患者相比，发生TTP的患者年龄几乎大三倍（P < 0.001），女性的可能性是前者的两倍多（P = 0.001）。由于超过90%的患者是UD骨髓受者，我们随后将20例接受UD骨髓移植（BMT）的患者与60例随机选择的未发生TTP的UD - BMT患者进行了比较。单因素分析显示，年龄、女性性别以及Ⅱ - Ⅳ级急性移植物抗宿主病（GvHD）再次成为显著的危险因素（P = 0.002），并且与慢性GvHD存在关联趋势（P = 0.083）。然而，经过逻辑回归分析后，只有年龄和性别仍然具有显著性（分别为P < 0.001和0.009）。我们报告22例患者中有86%死亡，仅3例存活，其中1例仍有血小板减少且依赖红细胞输血，这可能部分是由于移植物发育不全。17例患者中有6例对血浆置换有反应，但他们中的大多数最终因TTP死亡，通常与GvHD或真菌感染有关。
