作者：吴国光 周燕  钟周琳  李丽兰  刘金莲  申卫东
来源：中国输血杂志, 2014, 27(1):18-21.

摘要：目的 对CD36（GPⅣ）同种抗体介导血小板输注无效（PTR）的4例中国广西病例作确诊实验。 方法 应用血小板流式细胞荧光免疫检测技术（PIFT-FC）和血小板抗原单克隆抗体特异性免疫固定试验（MAIPA）检测及鉴定4名临床PTR患者血清中的抗-HPA和抗-CD36；以PIFT-FC试验和单核细胞检测流式细胞荧光免疫检测技术（MIFT-FC）检测CD36抗原在患者血小板及单核细胞上的表达情况和作CD36缺失的表现型分型。对患者CD36作基因序列分析和以DNA 为基础的PCR-SSP技术鉴定并确认CD36基因突变点，以证实患者CD36的缺失。 结果 4名PTR患者体内都含抗-CD36且均为CD36 Ⅰ型缺失者，患者CD36基因检测分别为1例380C>T、2例329-330 del AC和1例1156C>T基因突变。 结论 实验确诊4例PTR均为抗-CD36介导；提示在临床遇到PTR病例时，鉴于中国人群有较高的CD36缺失率的特征，除考虑HPA及HLA等的免疫因素外，应对抗-CD36介导的同种免疫反应而产生的血小板免疫异常予以重视，并给予针对性的诊断和治疗。
关键词：CD36缺失；血小板输注无效; 同种免疫反应；同种抗体；基因突变

Experimental Study on the Platelet Transfusion Refractoriness Mediated by the Anti-CD36 Antibodies--- Four Cases Reports in China

WU Guo-Guang ,  ZHOU Yan, ZHONG Zhou-Lin, LI Li-Lan, LIU Jin-Lian, SHEN Wei-Dong
Chinese Journal of Blood Transfusion, 2014, 27(1):18-21.

Abstract  Objective：Experimental study on the platelet transfusion refractoriness (PTR) mediated by the anti-CD36 antibodies and description of four cases in China. Methods: Four patients with clinical PTR were studied.  The anti-Human Platelet Antigens (HPA)  antibodies and anti-CD36 antibody in the sera of four patients were detected and identified by Platelet Immunofluorescence Test-Flow Cytometry（PIFT-FC）and Monoclonal Antibody-specific Immobilization of Platelet Antigens Assay (MAIPA). CD36 expression on platelets and monocytes were examined by IFT-FC and Monocyte Immunofluorescence Test-Flow Cytometry (MIFT) . For the confirmation of CD36 deficiency, the CD36 Gene of the four patients was sequenced as genomic analysis, and their mutations of the CD36 Genes were further verified by DNA-based PCR-SSP assay. Results: All four patients were Type I CD36 deficiency and the anti-CD36 antibodies were identified in patients’ sera. CD36 mutations identified in 4 patients were 380C>T(1 case), 329-330 del AC(2 cases) and 1156C>T (1 case), respectively.  Conclusion: The patients with platelet transfusion refractoriness were approved that caused by anti-CD36 antibodies. Since Chinese populations have a higher proportion of CD36 deficiency, isoimmunization against CD36 should be considered in patients with apparent alloimmune platelet disorders not explained by immunization against HPA and HLA. Such cases should give targeted diagnosis and treatment.
Key words: CD36 Deficiency; Platelet Transfusion Refractoriness; Isoimmunization; Isoantibody; Gene Mutation;