Acquired antithrombin type IIb deficiency after liver transplantation: a case report.
A 3-year-old girl with multifocal hepatoblastoma was referred to our clinic for living-donor liver transplantation, the patient's father being the donor. Pretransplant evaluation revealed that the father presented partial asymptomatic antithrombin (AT) deficiency, with no inherited AT deficiency found in the girl. The genetic testing showed an AT type IIb deficiency responsible for a defect in the heparin-binding region of AT which is less thrombogenic but more common than the other AT qualitative defects. Her mother was ABO incompatible. Despite the thrombophilia on the father's side, transplantation was successfully performed under replacement therapy with intravenous AT concentrate and low-molecular-weight heparin thromboprophylaxis given to both the recipient and the donor. No thrombotic complications occurred. In the posttransplantation course, acquired partial AT deficiency was detected in the recipient, who received adjuvant chemotherapy without thrombotic complications. This case report highlights the relevance of full thrombophilic work-up before liver transplantation from a living donor, while illustrating that the procedure can be successfully performed in the case of AT deficiency on the donor's side provided that appropriate AT supplementation and thromboprophylaxis are administered to both the recipient and the donor.
Pediatric gastroenterology Unit             Pediatric Surgery and Transplant Unit             Hemostasis and Thrombosis Unit, Division of Adult Hematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
L
L
Hougardy
X
X
Stephenne
R
R
Reding
E
E
Sokal
F
F
Smets
C
C
Lambert
C
C
Hermans
D000925:Anticoagulants / Q000627:therapeutic use; D020152:Antithrombin III Deficiency / Q000188:drug therapy / Q000209:etiology*; D002675:Child, Preschool; D005260:Female; D020022:Genetic Predisposition to Disease; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D018197:Hepatoblastoma / Q000601:surgery*; D006801:Humans; D008113:Liver Neoplasms / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects*; D019520:Living Donors; D008297:Male; D019851:Thrombophilia / Q000517:prevention & control
D000925:Anticoagulants; D006495:Heparin, Low-Molecular-Weight