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.
Department of Pediatrics, National Defense Medical College, Saitama, Japan. haruki-komatsu@chive.ocn.ne.jp
H
H
Komatsu
A
A
Inui
T
T
Sogo
T
T
Fujisawa
H
H
Egawa
K
K
Tanaka
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