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Open access

Aneta Neskoromna-Jędrzejczak, Katarzyna Bogusiak, Krzysztof Chojnowski, Marta Robak and Jacek Treliński

Abstract

The preparation of patients with hemophilia before surgical operations and dental procedures constitutes a significant clinical challenge. This article presents the implantoprosthetic rehabilitation of a patient with severe hemophilia B (factor IX activity <1%). The patient was prepared for the surgical procedure with recombinant factor IX concentrate (Rixubis) during the clinical surgery study. Tooth extraction and the implantation of four dental implants in the mandible were planned: one dental implant of 3.7 mm diameter and 10 mm length in the place of tooth 35, and another of 3.2 mm diameter and 10 mm length in the place of tooth 37. The next two implants were implemented 1 month later: one implant 3.7 mm in diameter and 10 mm in length in the place of tooth 46, and another implant 3.2 mm in diameter and 10 mm in length in the area of tooth 44. Appropriate substitution of the missing coagulation factor, together with the use of local hemostatic therapy, allowed dental implantation to be performed without excessive blood loss in this patient with severe hemophilia B.

Open access

Omar El Graoui, Saadia Faez and Bouchra Oukkache

Abstract

Acquired hemophilia A (AHA), is a rare bleeding disorder with an incidence of approximately 1 per million/year.The disease is caused by the development of autoantibodies directed against clotting factor VIII and associated with an increased morbidity and mortality. In 50% of cases, FVIII autoantibodies occur in patients lacking any relevant concomitant disease; the remaining cases may be associated with postpartum period, autoimmune diseases, underlying hematologic or solid cancers, and infections.

We report the case of a 32-years-old woman, admitted in hemorrhagic shock, following a non-reversible bleeding after dental extraction. The Laboratory tests prolonged activated prothrombin thromboplastin time (APTT at 54s), and normal Prothombine time (PT at 75%). The mixing test revealed the presence of a circulating anticoagulant with a Rosner index at 15. Factors specific to the intrinsic pathway determined that the patient had a factor VIII deficiency. Acquired hemophilia was diagnosed.

At the local level, CT scan of the face objectified a right maxillary osteitis without sinus damage.The patient received transfusion of Red Blood Cells units and FFP (fresh frozen plasma) concentrates.but kept bleeding until an antibiotic treatment and corticosteroids (60mg/d) were added.

Open access

Sarah Gilgunn

Abstract

Clotting factor replacement therapy has proven a highly effective means of treating haemophilia A and B. But treatment involves frequent and lifelong infusion of factor concentrates and is generally prophylactic rather than curative. It is also extremely expensive, associated with inhibitor formation and does not fully abolish the potential for spontaneous bleeding. Gene therapy offers a potential cure for haemophilia, with the possible continuous expression of a clotting factor gene following the administration of a viral vector carrying the appropriate gene. Recent clinical trials of gene therapy for haemophilia have proven positive in selected patients and new studies are underway.

Open access

Liesbeth Schrijvers, Martin Bedford, Petra Elfvinge, Karin Andritschke, Bert Leenders and Christine Harrington

Abstract

Background: A broad scoping exercise was undertaken to assess and quantify haemophilia nursing care in Europe. Methods: A web-based survey in English was sent to known networks of haemophilia nurses working in Europe. This survey included questions concerning the haemophilia treatment centre, educational level, work activities, gaining knowledge/ expertise and development in the future. Results: In total, 94 nurses in 14 countries in Europe completed the survey. Overall, the majority (62%) of the nurses had over 20 years’ nursing experience, with 44% having more than 10 years’ experience in haemophilia. The educational level varied; with highest educational level of attainment being 41% at nondegree level nursing entry qualification, 35% BSc and 24% MSc. 21% worked in a centre where they treat only children, 26% only adults and 53% both. All had good access to treatment. The core activities (rated >80%) of a haemophilia nurse were: prepare and administer medication, venepuncture and CVADuse (except cannulation), providing education and telephone advice, coordination of (multidisciplinary) care and assistance with clinical trials. Furthermore, 35% stated that they initiated and performed nurse-led research. In the future, almost all nurses would like to develop their expertise and knowledge base by studying at Masters level and above, to have more responsibility, and to conduct research. Conclusion: This scoping exercise provides a baseline assessment of haemophilia nursing across Europe. The results may provide a basis for a more thorough investigation of the current role and the principles of haemophilia nursing care; future opportunities, and the training requirements to develop the specialty.

Open access

David Hopper

Abstract

A 73-year-old woman presented with recent onset significant spontaneous bruising. She also had a recent diagnosis of type 2 diabetes mellitus and was undergoing investigations for biochemical hyperthyroidism. A diagnosis of acquired haemophilia A was confirmed with laboratory testing in addition to thyrotoxic Graves’ disease. She had significant bruising of her left thigh and restricted mobility of the left leg. A significant left vastus lateralis haematoma was diagnosed by ultrasound scan. A course of FEIBA (Factor Eight Inhibitor Bypassing Activity) was commenced and physiotherapy input provided. Kinesio tape was applied to the thigh using a lymphatic drainage technique with the aim of reducing swelling and bruising. Eight days after the initial application of tape, bruising had significantly reduced and her range of movement had returned to normal. This case history demonstrates how kinesio tape may be used as a useful adjunct to standard therapy.

Open access

Takeshi Sugimoto, Kazuhide Morimoto, Hiromi Hashimoto, Yukie Kaneda, Shinya Ohata and Yoshiro Yasutomo

Abstract

An 88-year-old female was admitted with autoimmune hemolytic anemia (AIHA). Coagulation test revealed severe prolongation of activated partial thromboplastin time (APTT). APTT cross-mixing test with patient plasma and normal plasma demonstrated an inhibitory pattern. Several intrinsic coagulation factor activities, particularly factor IX, showed remarkable decreases, and the inhibitor titers for coagulation factors VIII and IX were elevated. Although AIHA with existing antiphospholipid (aPL) antibodies was diagnosed initially, purpura developed on extremities intermittently during the clinical course. Considering the possibility of coexisting acquired hemophilia, APTT cross-mixing test with patient’s plasma and equal amount of the recombinant factor VIII product instead of normal plasma was performed. The APTT value on equal mixing samples with patient plasma and recombinant factor VIII product was decreased to within the normal range, and coagulation factor IX activity was restored. These results indicate that the recombinant factor VIII product had a neutralizing effect on aPL antibodies. We concluded that recombinant factor VIII product may lead to the repair of incorrect results from the APTT-dependent diagnostic system in the presence of aPL antibodies.

Open access

Andrzej B. Szczepanik, Wojciech P. Dąbrowski, Anna M. Szczepanik, Konrad Pielaciński and Wojciech Jaśkowiak

Abstract

In cirrhotic hemophilia patients bleeding from esophageal varices is a serious clinical condition due to congenital deficiency of clotting factors VIII or IX, decreased prothrombin synthesis and hypersplenic thrombocytopenia. In hemophiliac with high-titer inhibitor bypassing therapy is required with activated prothrombin complex concentrates (aPCC) or recombinant activated coagulation factor VII (rFVIIa). Doses and duration treatment with these agents following endoscopic treatment of esophageal varices have not been yet established.

Authors report the first case of a severe hemophilia A patient with high titer inhibitor (40 BU) treated with repeated injection sclerotherapy. The patient was admitted with symptoms of massive esophageal variceal hemorrhage ceased with emergency sclerotherapy. Bypassing therapy was administered with aPCC at initial dose of 72.5 U/kg and then with average daily dose of 162 U/kg through 5 days. To achieved a total eradication of esophageal varices the patient was then subjected to four elective sclerotherapy procedures. Two were covered by aPCC with daily dose of 120 U/kg and 145 U/kg for 4 and 3 days respectively and the following two procedures were covered by rFVIIa with the initial dose of 116 µg/kg and the next doses of 87 µg/kg administered every 3 hours in procedure day and every 4 hours on the next two days. During all procedures excellent hemostasis was achieved and no hemorrhagic or thromboembolic complications were observed. Bypassing regimen therapy with aPCC and rFVIIa we applied have been shown to be safe and effective in this patient subjected to sclerotherapy procedures.

Open access

Mike Holland

Abstract

Services for people with haemophilia in Uganda are poor, resulting in a very low number of confirmed diagnoses. Over the past year, the Haemophilia Foundation of Uganda (HFU) has engaged in a concerted and coordinated media campaign focusing on radio, television and social media promoting the message that haemophilia results in painful shortened lives but is a treatable condition if appropriate services are in place. At the end of 2015, this awareness campaign culminated in a patient-screening day, along with a patient information camp. In advance of the patient testing day, a team from London’s Great Ormond Street Hospital ran an educational workshop for staff from Mulago Hospital. The team also attended the testing day, taking clinical histories from those attending, and ran workshop sessions at the patient meeting. The physician-training day attracted obstetricians, paediatricians, nurses, dentists, physiotherapists, midwives and pharmacists. Delegates understood that to develop a haemophilia service in Uganda required capacity building, and awareness raising, initially at a central level but gradually moving out to the regions. Physicians in Uganda have now embarked on a journey to providing haemophilia care. The next steps are to build political engagement and to continue raising awareness among the population.

Open access

Peter Collins

Abstract

Advances in the understanding of haemophilia require effective collaboration, both at the national and international levels. Such collaborations are currently playing a major role in elucidating the natural history of acquired haemophilia, and also in clarifying the issue of product-related inhibitor development in previously untreated patients with haemophilia.

Open access

Susan Hook

Abstract

A case study is presented illustrating the everyday challenges faced by patients with haemophilia and inhibitors and how treatment with bypassing agents or just FEIBA prophylaxis can help to control bleeding and improve functional ability.