Frakking F N J, van de Wetering M D, Brouwer N, Dolman K M, Geissler J, Lemkes B, Caron H N, Kuijpers T W
Emma Children's Hospital, Academic Medical Center (AMC), F8-245 Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Eur J Cancer. 2006 May;42(7):909-16. doi: 10.1016/j.ejca.2005.10.027. Epub 2006 Mar 6.
Children with cancer often have fever during chemotherapy-induced neutropenia, but only some develop serious infectious complications. Mannose-binding lectin (MBL) deficiency might increase infection susceptibility in these children. MBL genotype and phenotype were prospectively determined in 110 paediatric oncology patients. During febrile neutropenia, MBL concentrations were measured longitudinally in time. MBL genotype and phenotype were correlated to clinical and laboratory parameters. Structural exon-1 MBL2 mutations and the LX promoter polymorphism lead to deficient MBL concentrations. The capacity to increase MBL concentrations during febrile neutropenia was associated with MBL2 genotype. Infectious parameters did not differ between MBL-deficient and MBL-sufficient neutropenic children (n = 66). In contrast, MBL-sufficient patients had a greater risk of Intensive Care admittance (Relative Risk 1.6, 95% Confidence Interval 1.3-2.0, P = 0.04). MBL-deficient neutropenic children did not have more severe infections. However, most patients (61%) were severely neutropenic (<100 cells/microL), compromising the opsonophagocytic effector function of MBL. MBL substitution might still be beneficial in patients with phagocytic activity.
癌症患儿在化疗引起的中性粒细胞减少期间常出现发热，但只有部分患儿会发生严重的感染并发症。甘露糖结合凝集素（MBL）缺乏可能会增加这些患儿的感染易感性。前瞻性地测定了110例儿科肿瘤患者的MBL基因型和表型。在发热性中性粒细胞减少期间，纵向测定MBL浓度。MBL基因型和表型与临床及实验室参数相关。外显子1结构MBL2突变和LX启动子多态性导致MBL浓度不足。发热性中性粒细胞减少期间增加MBL浓度的能力与MBL2基因型有关。MBL缺乏和MBL充足的中性粒细胞减少患儿（n = 66）之间的感染参数没有差异。相比之下，MBL充足的患者入住重症监护病房的风险更高（相对风险1.6，95%置信区间1.3 - 2.0，P = 0.04）。MBL缺乏的中性粒细胞减少患儿没有更严重的感染。然而，大多数患者（61%）存在严重的中性粒细胞减少（<100个细胞/微升），这损害了MBL的调理吞噬效应功能。MBL替代疗法对具有吞噬活性的患者可能仍然有益。
