儿童血液病血小板无效输注的机制和治疗

The treatment and mechanism of platelet transfusion refractoriness in pediatric hematological disorders

目的探讨儿童血液肿瘤患者血小板无效输注的分子学机制及治疗。方法回顾性分析2013年至2014年收治的4例血小板无效输注患儿的临床特征及实验室检查资料,评估血浆置换对儿童血小板无效输注的疗效。结果 4例患儿中男2例、女2例,年龄2岁-11岁9个月,其中3例为急性单核细胞白血病（M5）,1例为重型再生障碍性贫血（SAA）。1例M5患儿首次化疗后、2例M5患儿造血干细胞移植后发生血小板无效输注,1例SAA患儿入院时即表现为血小板无效输注。免疫磁珠流式液相芯片技术（Luminex）检测,3例患儿HLA-I类抗体阳性,1例患者HLA-I类阴性。血浆置换后,血小板无效输注得到明显改善。结论 HLA-I类抗体阳性与血小板无效输注密切相关,血浆置换术可治疗血小板无效输注。

Objective To explore the treatment and mechanism of platelet transfusion refractoriness in pediatric hematological disorders. Methods Retrospective analyses were performed from 4 children diagnosed with platelet transfusion refractoriness between 2013 and 2014 in Children＇s Hospital of Soochow University. Clinical manifestation, laboratory tests, and the effect of plasma exchange were evaluated on pediatric platelet refractoriness. Results Two males and two females with age ranged from 2 years to 11 years and 9 months met the criteria of platelet refractoriness. Three cases were diagnosed as AML-M5 （M5）, and one case was diagnosed as severe aplastic anemia （SAA）. Platelet refractoriness occurred in one case of M5 after induction chemotherapy, and two cases in the stage of hematopoietic stem cell transplantation （HSCT）. The SAA patient showed platelet refractoriness on her admission. The screening for HLA antibodies with the method of Immune Luminex beads streaming technology （Luminex） revealed that HLA-I antibody reaction were positive in three patients. All four patients showed a good response to the treatment of plasma exchange. Conclusions HLA-I class antibodies are closely associated with platelet refractoriness, the plasma exchange treatment is effective and safe for platelet refractoriness in children.