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异基因造血干细胞移植后儿科患者低丙种球蛋白血症的发病率及危险因素

Incidence and risk factors for hypogammaglobulinemia in pediatric patients following allo-SCT.

作者信息

Frangoul H, Min E, Wang W, Chandrasekhar R, Calder C, Evans M, Manes B, Bruce K, Brown V, Ho R, Domm J

机构信息

Division of Hematology/Oncology, Department of Pediatrics, Vanderbilt University, Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, TN, USA.

出版信息

Bone Marrow Transplant. 2013 Nov;48(11):1456-9. doi: 10.1038/bmt.2013.76. Epub 2013 May 27.

DOI:10.1038/bmt.2013.76
PMID:23708706
Abstract

We evaluated the incidence and risk factors for hypogammaglobulinemia after allogeneic hematopoietic SCT (HSCT) in pediatric patients. Ig levels were measured pre-transplant, every 2 weeks until day 100 and then monthly post SCT in 185 patients undergoing myeloablative HSCT. Median age was 9 years; 142 (77%) had malignant disease and 114 (62%) received stem cells from an unrelated source. Hypogammaglobulinemia (IgG <500 mg/dL) developed in 143 (77%) of the patients at a median of 56 days (range 15-339) post SCT. The cumulative incidence of hypogammaglobulinemia at 1 year was higher among patients who developed acute GVHD (97% vs 54%, P<0.001), and for those receiving stem cells from an unrelated source (94% vs 51%, P<0.001). The cumulative incidence of TRM was significantly higher for patients with hypogammaglobulinemia (P=0.026). In multivariable analysis, lower pre-transplant IgG level (P<0.001), younger age (P=0.012), diagnosis of malignant disease (P<0.001), receiving unrelated SCT (P<0.001) and development of acute GVHD (P<0.001) were all significantly associated with higher risk of hypogammaglobulinemia post HSCT. We conclude that hypogammaglobulinemia is common, following allogeneic HSCT in pediatric patients, especially in those with malignant diseases, those who receive an unrelated transplant or patients who develop GVHD.

摘要

我们评估了儿童患者异基因造血干细胞移植(HSCT)后低丙种球蛋白血症的发生率及危险因素。对185例行清髓性HSCT的患者在移植前、移植后每2周直至第100天以及之后每月测量免疫球蛋白(Ig)水平。中位年龄为9岁;142例(77%)患有恶性疾病,114例(62%)接受了来自非亲缘供者的干细胞。143例(77%)患者在HSCT后中位56天(范围15 - 339天)出现低丙种球蛋白血症(IgG<500mg/dL)。发生急性移植物抗宿主病(GVHD)的患者1年时低丙种球蛋白血症的累积发生率更高(97%对54%,P<0.001),接受非亲缘供者干细胞的患者也是如此(94%对51%,P<0.001)。低丙种球蛋白血症患者的移植相关死亡率(TRM)累积发生率显著更高(P = 0.026)。多变量分析显示,移植前IgG水平较低(P<0.001)、年龄较小(P = 0.012)、患有恶性疾病(P<0.001)、接受非亲缘HSCT(P<0.001)以及发生急性GVHD(P<0.001)均与HSCT后低丙种球蛋白血症的较高风险显著相关。我们得出结论,低丙种球蛋白血症在儿童患者异基因HSCT后很常见,尤其是患有恶性疾病、接受非亲缘移植或发生GVHD的患者。

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