BACKGROUND: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease and is a heterogeneous group of antibody deficiency syndromes characterized by hypogammaglobulinemia, recurrent bacterial infections, and frequent autoimmune manifestations. Liver transplantation (LT) is rarely performed in patients with CVID and associated end-stage liver disease. CASE REPORT: We report the successful results of 2 patients who underwent LT with pre-existing diagnosis of CVID. Case 1: A 21-year-old man affected by CVID and HCV-related cirrhosis underwent LT in December 2010 with a 67-year-old deceased donor liver graft. At the time of LT, MELD score was 30. The early post-LT course was characterized by a biliary stricture treated with Roux-en-Y repackaging of the anastomosis. Neither main infections nor acute rejection were detected during the postoperative period. After 43 months follow-up, the patient is alive and well with a histological recurrence of hepatitis C grade 1 and stage 2 according to Metavir staging. Case 2. On March 2013, a 53-year-old woman developed HBV-related fulminant liver failure and underwent urgent LT utilizing a 21-year-old deceased donor liver graft. The postoperative course was characterized by relaparotomy for hemoperitoneum. CMV infection was diagnosed 5 months after LT and resolved after valganciclovir therapy. After 6 months, mild acute rejection was diagnosed and treated with steroids. The patient is currently alive and well. The immunosuppressive regimen was based on Advagraf and early steroids discontinuation in both patients. CONCLUSIONS: LT should not be precluded to patients with CVID and end-stage liver diseases. Immunosuppression has a key role in this category of patients to balance the higher risk of rejection and serious infections.
BACKGROUND: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease and is a heterogeneous group of antibody deficiency syndromes characterized by hypogammaglobulinemia, recurrent bacterial infections, and frequent autoimmune manifestations. Liver transplantation (LT) is rarely performed in patients with CVID and associated end-stage liver disease. CASE REPORT: We report the successful results of 2 patients who underwent LT with pre-existing diagnosis of CVID. Case 1: A 21-year-old man affected by CVID and HCV-related cirrhosis underwent LT in December 2010 with a 67-year-old deceased donor liver graft. At the time of LT, MELD score was 30. The early post-LT course was characterized by a biliary stricture treated with Roux-en-Y repackaging of the anastomosis. Neither main infections nor acute rejection were detected during the postoperative period. After 43 months follow-up, the patient is alive and well with a histological recurrence of hepatitis C grade 1 and stage 2 according to Metavir staging. Case 2. On March 2013, a 53-year-old woman developed HBV-related fulminant liver failure and underwent urgent LT utilizing a 21-year-old deceased donor liver graft. The postoperative course was characterized by relaparotomy for hemoperitoneum. CMV infection was diagnosed 5 months after LT and resolved after valganciclovir therapy. After 6 months, mild acute rejection was diagnosed and treated with steroids. The patient is currently alive and well. The immunosuppressive regimen was based on Advagraf and early steroids discontinuation in both patients. CONCLUSIONS: LT should not be precluded to patients with CVID and end-stage liver diseases. Immunosuppression has a key role in this category of patients to balance the higher risk of rejection and serious infections.
Authors: Junmin Song; Ana Lleo; Guo Xiang Yang; Weici Zhang; Christopher L Bowlus; M Eric Gershwin; Patrick S C Leung Journal: Clin Rev Allergy Immunol Date: 2018-12 Impact factor: 8.667