Autoimmune Hemolytic Anemia Complicated by Portal Vein Thrombosis in a Pediatric Liver Transplant Recipient: A Case Report
Transplantation Proceedings, 2026 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Basım Tarihi: 2026
- Doi Numarası: 10.1016/j.transproceed.2026.07.044
- Dergi Adı: Transplantation Proceedings
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, EMBASE, MEDLINE, Academic Search Ultimate (EBSCO)
- Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Evet
Özet
Post-transplant autoimmune hemolytic anemia (AIHA) occurs in approximately 1% to 3% of pediatric liver transplant recipients. Its co-occurrence with portal vein thrombosis (PVT) is exceedingly rare and potentially life-threatening. We report a 10-month-old female who underwent living-donor liver transplantation for biliary atresia and presented with AIHA 5 months later. Laboratory evaluation revealed severe hemolytic anemia (hemoglobin 7.5 g/dL), markedly elevated lactate dehydrogenase (1751 IU/L), hyperbilirubinemia (total bilirubin 4.92 mg/dL), thrombocytopenia (75 × 103/μL), prolonged PT/INR, critically reduced Protein S activity (48.1%), and elevated D-dimer (3.78 mg/L; 7.6 × upper normal). A strongly positive Direct Antiglobulin Test confirmed AIHA. Abdominal computed tomography demonstrated hepatomegaly with portal vein thrombosis, confirmed by color-flow Doppler ultrasonography with turbulent, reduced flow. Tacrolimus dose was reduced and simultaneous high-dose methylprednisolone (4 mg/kg/d intravenously), rituximab, and therapeutic heparin were initiated. Complete portal vein recanalization was confirmed by Doppler on Day 5, with full immunologic remission (Direct Antiglobulin Test-negative) thereafter. This case demonstrates that post-transplant AIHA can precipitate PVT through a hemolysis-driven hypercoagulable cascade involving Protein S consumption, thrombocytopenia, and pro-coagulant erythrocyte microparticle release. Systematic coagulation profiling and Doppler ultrasonography at AIHA onset are mandatory in pediatric liver transplant recipients to prevent potentially fatal vascular complications.