Primary hypogammaglobulinemia complicated with liver cirrhosis and literature review

Objective To explore the pathogenesis, treatment and prognosis of primary hypogammaglobulinemia complicated with liver cirrhosis in a child. Method Pathogenesis, treatment and prognosis of X-linked agammaglobulinemia (XLA ) complicated with liver cirrhosis in a child were analyzed in Shanghai Children′s Medical Center.Using"primary hypogammaglobulinemia"and"liver cirrhosis"as keywords, literatures were searched from Pubmed and Chinese data of Weipu and Wanfang data from January 1988 to January 2015. Result The patient was a 12 years old boy with the chief complaint of 3 times hematemesis with diagnosis of XLA in the past 7 years. He received treatment with immunoglobulin (Ig) monthly for 6 years. He had no hepatitis C virus( HCV ) infection and serologic tests for autoantibodies were negative. Anti-HBs, anti-HBe and anti-HBc were positive, which revealed previous hepatitis B virus(HBV) infection. Gastroscopy suggested esophageal gastric varices. Liver pathology showed liver cell degeneration, necrosis, fiber tissue hyperplasia and pseudolobuli. After hospitalization the boy underwent liver transplantation (LT). He was given tacrolimus (3 mg/d), prednisone (5 mg/d), lamivudine (150 mg/d) and acyclovir (900 mg/d) by oral administration after LT. After 3 months follow-up, the boy was alive and well with stable results of liver function tests. There were no report in Weipu and Wanfang data. A total of 19 cases, including 12 cases of common variable immunodeficiency, 3 cases of XLA, 2 cases of Hyper-IgM syndrome and 2 cases of congenital hypogammaglobulinemia were obtained from Pubmed published between January 1, 1988 and January 1, 2015. Seventeen of the cases had HCV infection. Two cases had autoimmune hepatitis. Of the HCV infected patients, 15 were given intravenous gamma globulin. Seven of the 19 cases survived. Among 5 cases who received liver transplantation, 3 cases died. Conclusion In addition to HCV infection and autoimmune hepatitis as causes of liver cirrhosis in primary hypogammaglobulinemia, chronic HBV infection is another cause. Intravenous gammaglobulin is an important way of transmitting HCV and HBV infection. The effect of liver transplantation remains to be evaluated via further follow-up and studies. Key words: Immunologic deficiency syndromes; Liver cirrhosis; Child