Immunoglobulin therapy for successful management of prolonged, recurrent jaundice in a young adult male with combined immunodeficiency


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.

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The authors thank Shiro Watanabe, Isamu Hama and Hiroki Honda for their cooperation (Department of Gastroenterology and Hepatology, Shinrakuen Hospital). We thank the Initiative on Rare and Undiagnosed Diseases (IRUD) for their support.

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CO, TU AK, KT, KH, and AT: analysis of data and drafting of the manuscript; ST: critical revision of the manuscript.

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Correspondence to Atsunori Tsuchiya.

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The authors declare that they have no current financial arrangement or affiliation with any organization that may have a direct influence on their work. Chiyumi Oda, Atsunori Tsuchiya, Atsushi Kimura, Kentaro Tominaga, Kazunao Hayashi, Takashi Ushiki, Hajime Umezu and Shuji Terai declare that they have no conflict of interest.

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Oda, C., Tsuchiya, A., Kimura, A. et al. Immunoglobulin therapy for successful management of prolonged, recurrent jaundice in a young adult male with combined immunodeficiency. Clin J Gastroenterol (2021).

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  • Immunoglobulin G
  • Intravenous immunoglobulin
  • B-cell
  • T-cell
  • NK-cell