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首页> 外文期刊>Hong Kong Journal of Paediatrics >Successful Treatment of X-Linked Lymphoproliferative Disease (XLP) with Anti-CD20 Monoclonal Antibody (Rituximab) Followed by Mismatched Unrelated Cord Blood Transplantation
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Successful Treatment of X-Linked Lymphoproliferative Disease (XLP) with Anti-CD20 Monoclonal Antibody (Rituximab) Followed by Mismatched Unrelated Cord Blood Transplantation

机译：抗CD20单克隆抗体（Rituximab）成功治疗X连锁淋巴组织增生性疾病（XLP），然后进行不匹配的无关脐血移植

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A 4-year-old boy with X-linked lymphoproliferative disease (XLP) developed life-threatening acute lymphoproliferative crisis and failed to respond to conventional treatment of dexamethasone and etoposide. With the knowledge that uncontrolled alloreactive cytotoxic T-cell responses triggered by EBV-transformed B cells is the main cause of XLP, anti-CD20 monoclonocal antibody (Rituximab) which directed against B lymphocytes was use to damp down the patient's dysregulated immune response. He responded well to this novel approach and entered into complete remission with this treatment. His inherited immuno-deficient genetic defect was subsequently corrected by unrelated cord blood transplantation.
机译：一个四岁的男孩患有X连锁淋巴组织增生性疾病（XLP），发展为威胁生命的急性淋巴组织增生性危机，对地塞米松和依托泊苷的常规治疗无效。已知由EBV转化的B细胞触发的不受控制的同种异体反应性细胞毒性T细胞反应是XLP的主要原因，针对B淋巴细胞的抗CD20单克隆抗体（Rituximab）被用于减轻患者免疫反应失调的情况。他对这种新方法反应良好，并通过这种治疗完全缓解。他的遗传性免疫缺陷遗传缺陷随后通过无关的脐血移植得以纠正。

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