Successful liver transplant in a patient with common variable immunodeficiency concomitant with liver cirrhosis

In a 13 year old girlwith early stage of liver cirrhosis, chronic sinusitis and candidiasis, the common variable immunodeficiency (CVID) was diagnosed. Patient revealed decreased level of serum immunoglobulins, considerably decreased number of B-cells, CD4+ lymphocytes and impaired lymphocyte proliferative response to mitogens. The substitution treatment with intravenous immunoglobulins (IVIG) was introduced. During three years of observation good response to IVIG therapy without serious infections was noted, however the progression to end-stage liver failure occurred. At the age of 16 years the symptoms of liver failure exacerbated, with concomitant splenomegaly and ascites. The scintigraphy and liver biopsy confirmed the end-stage liver cirrhosis in due course of sclerosing cholangitis. patient underwent liver transplantation at age 17. At the time of liver transplantation, end to side Roux-en-Y choledochojejunostomy was constructed for biliary drainage. Since that time she receives both immunosuppressive (tacrolimus) and IVIG treatment and shows no clinical and biochemical symptoms of liver dysfunction or severe infections. In conclusion, we suggest that liver transplantation should also be considered as treatment of end-stage liver failure in patients with primary immunodeficiency.