title: Refractory autoimmune hemolytic anemia after intestinal transplant responding to conversion from a calcineurin to mTOR inhibitor.
abstract: AIHA is a rare and serious complication of solid organ transplantation. Herein, we report four cases of warm or mixed AIHA in pediatric patients following combined liver, small bowel and pancreas transplant. The hemolysis was refractory to multiple treatment modalities including steroids, rituximab, IVIG, plasmapheresis, cytoxan, discontinuation of prophylactic penicillin, and a change in immunosuppression from tacrolimus to cyclosporine. All patients had resolution or marked improvement of hemolysis after discontinuation of maintenance of CNI and initiation of sirolimus immunosuppression. One patient developed nephrotic syndrome but responded to a change in immunosuppression to everolimus. Three of the four patients continue on immunosuppression with sirolimus or everolimus without further hemolysis, evidence of rejection or medication side effects. Based on our experience and review of similar cases in the literature, we have proposed a treatment algorithm for AIHA in the pediatric intestinal transplant patient population that recommends an early change in immunosuppressive regimen from CNIs to sirolimus therapy.
authors.affiliation: Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE 68198-2168, USA.
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Acquazzino
authors.forename: Ryan T
authors.initials: RT
authors.lastname: Fischer
authors.forename: Alan
authors.initials: A
authors.lastname: Langnas
authors.forename: Don W
authors.initials: DW
authors.lastname: Coulter
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology / Q000628:therapy*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D019703:Calcineurin / Q000378:metabolism*; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D000068338:Everolimus; D005260:Female; D006461:Hemolysis; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007165:Immunosuppression Therapy / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007223:Infant; D007422:Intestines / Q000637:transplantation*; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D008499:Medical Records; D009404:Nephrotic Syndrome / Q000209:etiology; D016035:Pancreas Transplantation / Q000009:adverse effects*; D010406:Penicillins / Q000627:therapeutic use; D010956:Plasmapheresis; D012189:Retrospective Studies; D000069283:Rituximab; D020123:Sirolimus / Q000031:analogs & derivatives / Q000627:therapeutic use; D013256:Steroids / Q000627:therapeutic use; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors* / Q000378:metabolism*; D016559:Tacrolimus / Q000627:therapeutic use; D014180:Transplantation / Q000009:adverse effects; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D010406:Penicillins; D013256:Steroids; D000069283:Rituximab; D016572:Cyclosporine; D003520:Cyclophosphamide; D000068338:Everolimus; D058570:TOR Serine-Threonine Kinases; D019703:Calcineurin; D020123:Sirolimus; D016559:Tacrolimus
keywords: calcineurin inhibition; hemolytic anemia; intestinal transplant; mTOR inhibitor