PubMed · 10444294
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E A Von Willebrand. 1999. Hereditary pseudohaemophilia.. https://doi.org/10.1046/j.1365-2516.1999.00302.x
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All reported cases of non-catheter induced venous thrombosis in patients with hemophilia A or B have been carefully evaluated. A total of 27 cases were reported,12 patients with hemophilia A and 15 patients with hemophilia B. The age of patients varied between 9 and 67 years. There were 10 cases of deep vein thrombosis, 8 patients with pulmonary embolism accompanied or not by deep vein thrombosis, 5 cases of superficial vein thrombosis. In addition, there were 3 cases of thrombosis in unusual sites (1 retinal central vein thrombosis and 2 portal vein thrombosis). Finally, in one case, venous thrombosis was multiple. There was a fatality in a hemophilia B patient with pulmonary embolism. The most frequent risk or triggering factor in hemophilia A was the administration of Feiba or rFVIIa concentrates in patients with inhibitors. Surgery together with Prothrombin Complex concentrates was the most frequent cause in hemophilia B patients. Congenital associated prothrombotic risk factors were present in two patients. No or very few therapeutic procedures were initiated in these patients but for a suspension or reduction of concentrates infusion. In a few instances low molecular weight heparin was given for a few days. The frequent association of venous thrombosis with infusion of concentrates indicates the need for a careful evaluation of patients about to receive such therapy.
We describe an extremely rare case of subcapsular rupture of the liver associated with hemophilia A in a neonate. Although, the neonate was in good condition after birth, at 13 hours, he became pale and his abdomen distended. At 17 hours after birth, an emergency laparotomy was performed. The subcapsular rupture of the left side of the liver was found. The ruptured area was closed with a continuous suture, and additional mattress sutures with pledget provided satisfactory hemostasis. On the second day after the operation, relaparotomy was done because recurrent bleeding was suspected. There was no bleeding from the sutured liver. Oozing was found at the suture line on the abdominal wall. An additional postoperative observation was a decrease of factor VIII less than 1%, and hemophilia A was confirmed.