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慢性移植物抗宿主病的临床表现、诊断和分级

Author
Nelson J Chao, MD
Section Editor
Robert S Negrin, MD
Deputy Editor
Rebecca F Connor, MD
Translators
唐晓文, 主任医师，教授

引言

急性和慢性移植物抗宿主病(graft-versus-host disease, GVHD)为多系统疾病，是异基因造血干细胞移植(hematopoietic cell transplant, HCT)的常见并发症。来自不相合供者的免疫细胞(移植物)将移植受者(宿主)识别为“异己”，从而启动免疫反应导致移植受者发生疾病时，GVHD便发生。 (参见“移植物抗宿主病的发病机制”)

以发病时间在移植后100日为界，GVHD已被经典地分为急性和慢性。然而，目前发现在这些指定时间段之外可能发生急性和慢性GVHD的征象，因此这种传统分类方法受到了挑战。这一观察发现已使得人们更多地运用临床表现来区分急性和慢性GVHD，而非一个规定的时间段来区分。广为接受的美国国立卫生研究院(National Institutes of Health NIH)GVHD诊断共识标准中包括慢性GVHD和急性GVHD的诊断性或特征性表现同时出现的重叠综合征[1]。

慢性GVHD的临床表现包括类似于扁平苔藓或硬皮病皮肤表现的皮肤受累；口腔黏膜干燥伴溃疡和胃肠道硬化；以及血清胆红素浓度升高。而急性GVHD患者常表现出典型的斑丘疹；腹部痛性痉挛伴腹泻；以及血清胆红素浓度升高。 (参见“急性移植物抗宿主病的临床表现、诊断和分级”，关于‘临床和组织学表现’一节)

本专题将讨论慢性GVHD的临床表现、诊断和分级。慢性GVHD的治疗以及急性GVHD的相关问题参见其他专题。 (参见“慢性移植物抗宿主病的治疗”和“急性移植物抗宿主病的预防”和“急性移植物抗宿主病的临床表现、诊断和分级”)

流行病学

慢性GVHD可发生于既往急性GVHD之后或与之同时发生，或发生于无急性GVHD病史的患者中(如，新发疾病)。尚不明确异基因HCT后慢性GVHD的确切发病率。虽然大约40%的异基因HCT受者会发生慢性GVHD，但所报道的发病率为6%-80%，取决于是否存在危险因素及应用的诊断标准[1-4]。虽然已识别出发生GVHD的危险因素，但由于慢性GVHD的鉴别、测评和记录情况的差异性，目前尚未获得不同队列中GVHD发病率的可靠估计值。

                          

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Literature review current through: 2017-06 . | This topic last updated: 2016-08-15.
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