Severe hypogammaglobulinemia associated with hepatic vein stenosis causes cytomegalovirus infection after living-related liver transplantation.
Hepatic vein stenosis is a vascular complication that can lead to graft loss after liver transplantation. Although ascites frequently occurs as a symptom of hepatic vein stenosis, the development of severe hypogammaglobulinemia associated with hepatic vein stenosis has not been reported in the literature. An 8-year-old boy underwent living-related liver transplantation (LRLT) because of Wilson disease with chronic hepatic failure. Because de novo autoimmune hepatitis was diagnosed 1 year after LRLT, azathioprine, and prednisolone were added to the baseline immunosuppression of tacrolimus. The patient developed ascites with severe hypogammaglobulinemia (immunoglobulin G [IgG], 288 mg/dL) 2 years after LRLT. Ultrasonography and angiography disclosed stenosis of the hepatic vein. The ascites completely resolved after percutaneous balloon angioplasty. Despite serum IgG trough levels of >500 mg/dL maintained by the addition of immunoglobulin, cytomegalovirus reactivation and sepsis occurred. Serum IgG levels should be monitored to prevent opportunistic infections when hepatic vein stenosis is diagnosed after LRLT.
Komatsu
H
H
Department of Pediatrics, National Defense Medical College, Saitama, Japan. haruki-komatsu@chive.ocn.ne.jp
Inui
A
A
Sogo
T
T
Fujisawa
T
T
Egawa
H
H
Tanaka
K
K
D000361:Agammaglobulinemia / Q000209:etiology*; D001201:Ascites / Q000209:etiology / Q000276:immunology; D002648:Child; D003251:Constriction, Pathologic / Q000209:etiology / Q000276:immunology; D003586:Cytomegalovirus Infections / Q000188:drug therapy / Q000276:immunology*; D006503:Hepatic Veins; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D009894:Opportunistic Infections
D016756:Immunoglobulins, Intravenous