Thromboelastography in pre-surgery monitoring in Hemophilia A with high inhibitor titer: case report and literature review (original) (raw)
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Surgery in Hemophilia A Patients with Factor VIII Inhibitor: 10Year Experience
World Journal of Surgery, 1996
Patients with hemophilia A and circulating anticoagulant (factor VIII inhibitor) present a difficult, even unsolvable problem, particularly if they require surgical treatment and the inhibitor titer is high. During the 1986 -1995 period 29 surgical procedures on inhibitor hemophilia A patients were performed in our center. Each of the cases had an individual character, and all demanded special clinical treatment. Based on this experience we present the possibilities of hemostasis maintenance during the perioperative period with high doses of human or porcine factor VIII, aPCC, plasmapheresis, and extracorporeal antibody adsorption to protein A-Sepharose. In some patients hemostasis maintenance requires combined treatment. To induce immunotolerance in patients with inhibitor is the gold standard treatment because it is then possible to achieve proper hemostasis after factor VIII infusion. Various methods of immunotolerance induction have been discussed and compared with our experience with immunotolerance induction in 11 patients with small factor VIII doses (25 IU/kg twice a week) and the modified Malmö protocol in 15 patients.
Annals of Hematology & Oncology, 2019
Acquired Hemophilia (AH) poses several challenges to clinicians due to potential delays in diagnosis, based on a high index of suspicion, and a high risk of limb and life-threatening bleeding. We here report a case of AH with extremely high inhibitor titer (up to 1200 BU) in a patient who also developed venous thrombosis requiring anticoagulant treatment after prolonged immobilization for femur fracture. Multiple lines of immunosuppressive treatment were needed to achieve inhibitor eradication, probably due to the extremely high inhibitor titer, bleeding management also required several lines of treatment with bypassing agents. Bleeding treatment was here monitored by global hemostatic assays. Management of AH in a reference center allowed to achieve complete remission even in a very intricate condition.
Diagnostic evaluation of our patients with hemophilia A: 17-year experience
Türk Pediatri Arşivi, 2015
Aim: Hemophilia A is a rare inherited bleeding disorder resulting from factor VIII deficiency and is a group of diseases characterized by intra-articular and intramuscular bleeding. In this study, we aimed to retrospectively evaluate the treatment outcomes, demographic and clinical characteristics of our patients who were treated and followed up for last 17 years in our pediatric hematology unit with a diagnosis of Hemophilia A. Material and Methods: The medical records of 83 patients who were diagnosed with Hemophilia A and followed up between 1997 and 2014 in our hospital's pediatric hematology clinic were reviewed retrospectively. The demographic data, prophylaxis state, development of inhibitors and clinical characteristics of the patients were evaluated. Results: When the complaints at presentation were examined, it was found that 27 (32%) patients had hemarthrosis, 24 (29%) patients had ecchymosis and hematoma, 13 (16%) patients had prolonged bleeding after trauma or cut, 10 (12%) patients had gingival, mouth or nose bleeding, 4 (5%) patients had prolonged bleeding after circumcision, 4 (5%) patients had gastrointestinal bleeding, 1 (1%) patient had hematuria. Fifty (60%) patients were considered severe hemophilia A, 20 (24%) patients were considered moderate hemophilia A and 13 (16%) patients were considered mild hemophilia A according to factor activity. Among severe hemophilia A patients, primary prophylaxis was being administered in 2 (2%) patients and secondary prophylaxis was being administered in 40 (48%) patients. Inhibitor positivity was found in 8 (10%) of these patients. It is found that hemophilic artropathy developed in 17 patients and 8 of these 17 patients had undergone radioisotope synovectomy. Conclusions: Treatment of severe bleeding in hemophilia A patients should be performed in hospital and the presence of inhibitor must be investigated in cases of uncontrolled bleeding where adequate doses of factor concentrates have been administered for treatment. In order to decrease the development of inhibitor, prophlaxis should be suggested to patients rather than repetetive treatment when bleeding occurs. The radioactive synovectomy should not be overlooked in countries like ours in which factors can not be used adequately.
Surgery and inhibitor development in hemophilia A: a systematic review
Journal of Thrombosis and Haemostasis, 2011
Although the association between intensive treatment and the formation of inhibiting antibodies towards factor VIII (FVIII) in hemophilia A has been demonstrated, the contributing effect of surgery is presently unclear. The release of immunological danger signals resulting from tissue damage during surgery in the presence of a high FVIII antigen load may elicit the formation of FVIII antibodies. The aim of this systematic review was to investigate the role of surgery in the inhibitor risk associated with intensive treatment as compared with treatment for bleeding and prophylactic administration of FVIII. A comprehensive literature search was performed that identified four cohort studies and three case control studies, comprising 342 inhibitor patients among a total of 957 hemophilia A patients. Intensive treatment increased the inhibitor risk, most pronounced with intensive treatment of ≥ 5 exposure days (EDs) compared with < 3 EDs (OR, 4.1; 95% confidence interval, 2.6-6.5). Pooled odds ratio for inhibitor development in severe hemophilia patients that received intensive treatment for surgery at first exposure was 4.1 (95% confidence interval, 2.0-8.4) compared with treatment for bleeding or prophylaxis. Information on continuous infusion, previously treated patients and non-severe hemophilia A was insufficient for valid meta-analyses. Intensive FVIII treatment for surgery at first exposure leads to a higher inhibitor risk in hemophilia A patients compared with intensive treatment for bleeding.
Iranian Red Crescent Medical Journal, 2013
The development of inhibitors against administered clotting factors may render replacement therapy ineffective for some hemophilia patients. Such patients are therefore at the highest risk of developing arthropathy. Elective orthopedic surgery (EOS) in hemophilic patients having such inhibitors remains a rare, expensive, and difficult surgery, whose management represents a significant challenge. We report the case of a 35-year-old man with a severe form of hemophilia A (factor VIII < 1%), who was suffering from repetitive spontaneous hemarthrosis, especially in his knee joints that had consequently become more susceptible to bleeding. The patient had a history of high levels of factor VIII inhibitor (> 5.0 Bethesda Unit [BU]/ml) as shown by the factor VIII inhibitor assay; therefore, we began treatment with factor VIIa for his mildto-moderate bleeding (90 µg/kg intravenous bolus injections). The interval between injections varied with the severity of the hemorrhage in each bleeding episode. The inhibitor level reduced to 3.1 BU/ml after three months, to 1.6 BU/ml after six months, and disappeared completely after one year of treatment. We administered factor VIII at a dose of 50 IU/kg every eight hours during the first three post-operative days, then continued administration with a dose of 40 IU/kg every 12 hours for another four days, and observed a very good response to treatment with no bleeding. Recombinant activated factor VII (rFVIIa) is not an inhibitor-removal strategy, but an inhibitor-bypassing product. However, in our patient, the treatment of mild-to-moderate bleeding with short-term use of rFVIIa and no exposure to factor VIII caused a gradual reduction in the inhibitor level over a period of 1 year.
Acquired Hemophilia A: A Permanent Challenge for All Physicians
Medicines
Acquired hemophilia A (AHA) is a rare disease with a prevalence in Europe of 1.5 per million. This diagnosis is significantly delayed in about one-third of all cases, leading to deferred treatment. The main signs of AHA are spontaneous bleeding seen in about two-thirds of all patients. AHA can be lethal in 20% of all symptomatic cases. This patient population’s main standard laboratory finding is a prolonged aPTT (activated prothrombin Time) with otherwise normal coagulation results. In addition, antibodies against FVIII (in Bethesda Units) and a quantitative reduction of FVIII activity are necessary to confirm AHA. The therapy of acute bleeding related to AHA is based on the following main principles: Pharmacologic control of the bleeding is of absolute importance. It can be achieved by administering either recombinant activated FVIIa “bypass therapy”; activated prothrombin complex; or Emicizumab, a bispecific monoclonal antibody. Eradication of the FVIII antibodies should be initi...
Consensus recommendations for the diagnosis and treatment of acquired hemophilia A
BMC Research Notes, 2010
Background: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by an autoantibody to coagulation factor (F) VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute, life-threatening hemorrhage, with 9-22% mortality, to mild bleeding that requires no treatment. AHA usually presents to clinicians without prior experience of the disease, therefore diagnosis is frequently delayed and bleeds under treated.
Perioperative management of persons with hemophilia (PWH) is challenge for surgeons and hematologists. A reduction of the mortality rate and complication was achieved since improvement of hemostatic controls using clotting factor concentrates (CFCs). However, dosing of CFC administration for the procedures is disagreed. The aim of the study was to evaluate the outcome of PWH without inhibitors undergoing invasive or surgical procedures. A total of 161 procedures, including 57 major and 104 minor ones were retrospectively reviewed. The characteristics of PWH, age at procedure, duration and total amount of CFC administration during perioperative period, hemostatic adequacy and complication were summarized. The study showed a low rate of bleeding (1.2 %), infection (0 %), thromboembolic event (0 %) and inhibitor development (0 %). And, it represented doses and duration of CFC administration for several major and minor procedures which could achieve excellent hemostatic control.