Successful treatment of autoimmune and lymphoproliferative complications of patients with intrinsic B-cell immunodeficiencies with Rituximab
Successful treatment of autoimmune and lymphoproliferative complications of patients with intrinsic B-cell immunodeficiencies with Rituximab
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DOI：
10.1111/j.1365-2141.2009.07987.x
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发表时间：
2010-02-01
影响因子：
6.5
通讯作者：
Hansen, Gesine
中科院分区：
文献类型：
--
作者：
Hennig, Christian;Baumann, Ulrich;Hansen, Gesine
异质组原发性免疫缺陷需要个性化的诊断和治疗，以达到每个患者的最佳结果。两例具有内在b细胞类型转换缺陷（Hyper-IgM综合征亚类）的患者就是例证，由于缺乏准确的诊断，多年来淋巴细胞增殖和自身免疫决定了临床病程。基于遗传学或一种新的功能诊断方法，为每位患者建立了明确的个体诊断，并开始了利妥昔单抗治疗。观察期间（3-4年），自身免疫现象和全身性淋巴结病消失并控制良好，无不良反应。两名患者的生活质量均显著提高。
P>The heterogeneous group of primary immunodeficiencies requires personalized diagnosis and therapy to acheive an optimal outcome for each patient. This was exemplified by two patients with intrinsic B-cell class-switch defects (subclass of Hyper-IgM syndromes), where lymphoproliferation and autoimmunity determined the clinical course for many years due to lack of exact diagnosis. Based on genetics or a novel functional diagnostic approach, a definite individual diagnosis was established for each patient and they started Rituximab therapy. Autoimmune phenomena and generalized lymphadenopathy disappeared and remained well controlled during the observation period (3-4 years) without adverse effects. Quality of life increased remarkably in both patients.