Antibody‐mediated rejection after ABO‐incompatible pediatric living donor liver transplantation for propionic acidemia: A case report

Abstract

We herein present the case of a four-yr-old boy with PA who developed AMR after ABO-incompatible LDLT despite undergoing B cell desensitization using rituximab. Although the CD19+ lymphocyte count decreased to 0.1% nine days after the administration of rituximab, he developed a high fever which was accompanied by arthralgia due to a streptococcal infection 13 days after rituximab prophylaxis. After the clearance of the infection, he underwent ABO-incompatible LDLT 36 days after the administration of rituximab. The CD19+ lymphocyte count just prior to LDLT was 1.2%. He developed AMR five days after LDLT, and the antidonor-type IgM and IgG antibody titers increased to 1:1024 and 1:1024, respectively. He was treated by plasma exchange, IVIG, steroid pulse therapy, and rituximab re-administration; however, his liver dysfunction continued. Despite intensive treatment, he died due to complicated abdominal hernia, acute renal failure, and ARDS. This case suggests that a streptococcal infection may induce the activation of innate immune responses; thus, additional desensitization therapy should be considered prior to ABO-incompatible LDLT if B cell reactivation is suspected.

Long-term Outcomes of ABO-incompatible Pediatric Living Donor Liver Transplantation

“…As previously reported, case 9 developed streptococcal infection 13 days after rituximab, and LDLT was postponed. 19 CD19+ lymphocyte count decreased to 0.1% at 9 days after rituximab administration, but the number just before LDLT increased to 1.2%. The patient showed an increased ascites, a marked increase of hepatic enzyme levels, and decreased platelet levels on POD 5.…”

“…Case 9 showed specific clinical course suggesting the streptococcal infection after rituximab administration resulted in reactivation of B cells, which might trigger AMR. 19 In the light of experience, we think that additional desensitization therapy should be considered if the reactivation of B cells is suspected before ABOi LDLT.…”

Liver Transplantation in Children With Propionic Acidemia: Medium‐Term Outcomes

“…Since 1992, 49 patients (56 grafts) with PA and LT have been reported (Table ) . The median age at transplant was 3 years with a median follow‐up of 2.5 years (range, 0.01‐21 years).…”

Immunological Memory and Affinity Maturation After Vaccination in Patients With Propionic Acidemia

“…PCC catalyzes the conversion of propionyl-CoA to methylmalonyl-CoA, which enters the Krebs cycle via succinyl-CoA and acts as anaplerotic substance. The enzymatic defect leads to a disrupted activity of PCC resulting in the accumulation of propionic acid and related metabolic products (e.g., propionyl CoA, methylcitrate, propionylcarnitine, propionic acid) (1,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) (Figure 1). Abnormal amounts of metabolic products induce intoxication which lead to either acute or chronic symptoms.…”

“…The long-term sequelae mainly involve the heart and the CNS, with neurological symptoms (associated with progressive encephalopathy of varying severity), gastrointestinal symptoms (failure to thrive, anorexia), hematological (neutropenia, pancytopenia), and cardiac complications (prolonged QTc interval, arrhythmias, dilated or hypertrophic cardiomyopathy, heart failure) (1, 4-14, 16, 17). At any age, catabolic situations (e.g., during febrile episodes) can lead to acute metabolic crises associated with lifethreatening events (1,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”