Immunoglobulin therapy for successful management of prolonged, recurrent jaundice in a young adult male with combined immunodeficiency
Immunoglobulin therapy for successful management of prolonged, recurrent jaundice in a young adult male with combined immunodeficiency
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免疫球蛋白治疗成功治疗患有联合免疫缺陷的年轻成年男性的长期、复发性黄疸
DOI：
10.1007/s12328-021-01347-0
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发表时间：
2021
影响因子：
1
通讯作者：
Terai Shuji
中科院分区：
文献类型：
--
作者：
Oda Chiyumi;Tsuchiya Atsunori;Kimura Atsushi;Tominaga Kentaro;Hayashi Kazunao;Ushiki Takashi;Umezu Hajime;Terai Shuji
在与胆管消失综合征相关的药物性肝损伤中，Jaurine可能是持久性的。然而，胆汁回流恢复后复发性黄疸是不典型的。在这里，我们报告一个28岁的男子与长期，复发性黄疸（超过300天）和联合免疫缺陷（CID）的B细胞，T细胞和自然杀伤（NK）细胞。在住院期间观察到低丙种球蛋白血症，外周血流式细胞术检测到少量B细胞（2%的CD 19+细胞和2%的CD 20+细胞）。我们进一步检测了T细胞和NK细胞的功能障碍。根据这些发现，诊断为CID。我们推测低丙种球蛋白血症与黄疸有关。定期注射静脉注射免疫球蛋白（IVIG）后，粪便颜色逐渐变为棕色。然而，随着IgG水平降低，颜色恢复为白色。另一次IVIG给药后重复棕色至白色粪便模式，表明患者的血清免疫球蛋白水平与黄疸相关。随访时，每2 - 3周进行IVIG，总胆红素逐渐改善。免疫球蛋白替代治疗可作为黄疸合并CID的治疗选择之一。
Jaundice may be persistent in drug-induced liver injury associated with vanishing bile duct syndrome. However, recurrent jaundice is atypical, following bile flow restoration. Here, we report a 28-year-old man with prolonged, recurrent jaundice (more than 300 days) and combined immunodeficiency (CID) of B-cells, T-cells, and natural killer (NK) cells. Hypogammaglobulinemia was observed throughout his hospitalization, and peripheral blood flow cytometry detected a few B-cells (2% of CD19 + cells and 2% of CD20 + cells). We further detected the dysfunction of T-cells and NK cells. Based on these findings, CID was diagnosed. We presumed that hypogammaglobulinemia was related to the jaundice. After regular injections of intravenous immunoglobulin (IVIG), the stool color gradually turned brown. However, the color returned to white as IgG levels decreased. The brown-to-white stool pattern was repeated with another IVIG administration, suggesting that the patient’s serum immunoglobulin levels were related to the jaundice. On follow-up, IVIG was performed every two to three weeks, and his total bilirubin improved gradually. Immunoglobulin replacement therapy could be one of the treatment choices for jaundice with CID.