ABSTRACT
Acquired hemophilia A (AHA) is a very rare disease of idiopathic type, or secondary to neoplastic disease, the use of some drugs, or pregnancy. Owing to the rarity of the disease, no comparative clinical studies are currently available on the results of different intraoperative and postoperative pharmacological preventive strategies in arthroplasty surgery. We report the first case of a total hip replacement procedure for a fracture of the femoral neck performed in a patient affected by AHA. Treatment with recombinant factor VII was administered, at the dosage of 170 µg/kg preoperatively and continued postoperatively at 120-90 µg/kg every 2-6 h. The outcome described supports the use of the protocol adopted, demonstrating that the treatment duration was well adapted to this highly invasive surgical procedure.
Subject(s)
Arthroplasty, Replacement, Hip , Hemophilia A , Factor VII/administration & dosage , Factor VII/therapeutic use , Femur/surgery , Fractures, Bone/surgery , Humans , Perioperative CareABSTRACT
Data from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P < .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and inhibitor in a Cox regression model (HR, 2.65; 95% CI, 1.62-4.33; P < .001). The survival analysis of time to joint arthroplasty in the subset of patients with severe HA was not affected by the severity of factor VIII (FVIII) gene mutations. A systematic review of literature articles reporting joint arthroplasties in HA and HB showed that the proportion of HA patients who had undergone arthroplasties was higher than that of HB patients, in agreement with the findings in our Italian cohort. These data suggest that the 2 inherited coagulation disorders have a different severity of clinical phenotype.
Subject(s)
Arthroplasty/statistics & numerical data , Hemophilia A/surgery , Hemophilia B/surgery , Joint Diseases/epidemiology , Joint Diseases/surgery , Severity of Illness Index , Adolescent , Adult , Algorithms , Blood Coagulation Disorders/classification , Blood Coagulation Disorders/complications , Blood Coagulation Disorders/epidemiology , Blood Coagulation Disorders/surgery , Child , Cohort Studies , Hemophilia A/complications , Hemophilia A/epidemiology , Hemophilia B/complications , Hemophilia B/epidemiology , Humans , Italy/epidemiology , Joint Diseases/complications , Joint Diseases/etiology , Middle Aged , Retrospective Studies , Risk Factors , Young AdultABSTRACT
Heparin-induced thrombocytopenia is a serious complication during antithromboembolic prophylaxis caused by anti-heparin/platelet factor 4 (PF4) complex antibodies. It typically arises 3 to 15 days after the beginning of therapy and may result in an increased risk of arterial and venous thromboembolism. This article presents a case of thrombocytopenia associated with vein thrombosis in a man who underwent a total knee arthroplasty (TKA).
