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In conclusion, various complications might occur following arthroplasty in patients with haemophilia, including haemorrhage/haematoma, generation of the coagulation factor inhibitors, infection and prosthesis looseness. However, with haematologists’ cooperation, proper coagulation factor replacement therapy and skilled surgical technique, arthroplasty is possible and safe, and the rate of complication was similar to or less than that the literatures reported.

College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing China, for their assistance editing the manuscript.

Author contributions The manuscript was principally written by Jiliang Zhai and Xisheng Weng. The clinical data were collected by Jiliang Zhai, and the other coauthors.

Disclosures Acknowledgements We thank Hao Liu, from Mayo Clinic Rochester, MN, USA, and Wei Wang, from Department of Orthopaedic Surgery, Peking Union Medical

References 1 Rodriguez-Merchan EC. Total joint arthroplasty: the final solution for knee and hip when synovitis could not be controlled. Haemophilia 2007; 13(Suppl. 3): 49–58. 2 Wang K, Street A, Dowrick A, Liew S. Clinical outcomes and patient satisfaction following total joint replacement in haemophilia – 23-year experience in knees, hips and elbows. Haemophilia 2012; 18: 86–93. 3 Cohen I, Heim M, Martinowitz U, Chechick A. Orthopaedic outcome of total knee replacement in haemophilia A. Haemophilia 2000; 6: 104–9. 4 Sikkema T, Boerboom AL, Meijer K. A comparison between the complications and

The authors stated that they had no interests which might be perceived as posing a conflict or bias.

long-term outcome of hip and knee replacement therapy in patients with and without haemophilia; a controlled retrospective cohort study. Haemophilia 2011; 17: 300– 3. 5 Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. Haemophilia 2012; 18: 8–16. 6 Solimeno LP, Mancuso ME, Pasta G, Santagostino E, Perfetto S, Mannucci PM. Factors influencing the long-term outcome of primary total knee replacement in haemophiliacs: a review of 116 procedures at a single institution. Br J Haematol 2009; 145: 227–34. 7 Rahme M, Ehlinger M, Faradji A et al. Total knee arthroplasty in severe haemo-

philic patients under continuous infusion of clotting factors. Knee Surg Sports Traumatol Arthrosc 2012; 20: 1781–6. 8 Silva M, Luck JV Jr. Long-term results of primary total knee replacement in patients with hemophilia. J Bone Joint Surg Am 2005; 87: 85–91. 9 Wong JM, Mann HA, Goddard NJ. Perioperative clotting factor replacement and infection in total knee arthroplasty. Haemophilia 2012; 18: 607–12. 10 Goddard NJ, Mann HA, Lee CA. Total knee replacement in patients with endstage haemophilic arthropathy: 25-year results. J Bone Joint Surg Br 2010; 92: 1085–9.

Endovascular Abdominal Aortic Aneurysm Repair in a mild Haemophilia A Patient: a Case Report P . G A U T I E R , * † Y . R E P E S S E , * † E . J O G U E T , ‡ X . T R O U S S A R D , * A . B O R E L - D E R L O N * † and L. BERGER‡ *Biological hematology department, CHU Caen, Caen, France; †Hemophilia Centre, CHU Caen, Caen, France; and ‡Vascular surgery department, CHU Caen, Caen, France

Haemophilia A is a genetic disorder resulting from a deficiency of factor VIII (FVIII), a protein necessary for normal blood clotting. Epidemiological data report a dramatic improvement in haemophilia care over the last 50 years, and the life expectancy of haemophilia patients now approaches that of the general population [1]. Yet, evidence-based guidelines for the acute Correspondence: Philippe Gautier, CHU de Caen, Departement d’hematologie biologique, Avenue de la C^ ote de Nacre, 14033 Caen Cedex, France. Tel.: +33 2 31 06 53 31; fax: 0033231065099; e-mail: [email protected] Accepted after revision 15 January 2015 Haemophilia (2015), 21, e223--e259

management and secondary prophylaxis (FVIII treatment, antithrombotic and antiplatelet therapies) of cardiovascular diseases in haemophilia patients are still lacking, and very little is known about how to handle the increased risk of bleeding associated with invasive procedures and antithrombotic agents. We herein report the surgical and haematological strategies used in a mild haemophilia A patient who successfully underwent endovascular aortic aneurysm repair (EVAR). A 63-year-old patient was referred for treatment of an aortic abdominal aneurysm and right common iliac aneurysm that a CT scan performed to investigate lower back pain had fortuitously detected. The patient © 2015 John Wiley & Sons Ltd

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Fig. 1. Preoperative CT scan 3D reconstruction (CT: coeliac trunk, SMA: superior mesenteric artery, LRA: left renal artery, RRA: right renal artery, AAA: aortic abdominal aneurysm, CIA: common iliac artery, CIAA: common iliac artery aneurysm, EIA: external iliac artery, IIA: internal iliac artery).

had first been diagnosed with mild haemophilia A (FVIII:C at 6 IU/dL) at the age of 30 years during a preoperative check-up for plastic surgery. A missense mutation was identified in F8 gene: exon 23, c.6506G>A, p.Arg2169His. This mutation has been previously associated with FVIII inhibitor development [2]. He has since undergone several infusions with clotting factors, first with plasma-derived FVIII, followed by recombinant FVIII. In 1998, following substitution therapy with immunopurified human FVIII for multiple dental extractions, the patient developed a FVIII inhibitor that peaked at 2.5 Bethesda units per mL. An immune tolerance protocol (50 IU/kg per day for 3 months) was performed successfully. The patient © 2015 John Wiley & Sons Ltd

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has since undergone successful on-demand treatment for knee haemarthrosis and coronarography without FVIII inhibitor relapse. In 2008, he developed an acute coronary syndrome for which left anterior interventricular artery stenting was conducted. The patient has since been treated only with low-dose aspirin. When the CT scan detected the aortic abdominal (50 mm diameter) and right common iliac (30 mm diameter) aneurysms, the patient was considered high risk for open surgery, and an endovascular aneurysm repair (EVAR) was scheduled. Preoperative preparation comprised a substitution therapy consisting of 3000 IU (37 IU kg1) of recombinant FVIII concentrate, administered 15 min prior to the intervention so as to achieve full correction of FVIII activity (104%). Treatment was then repeated 12 h after the intervention, the residual FVIII level just prior to the second recombinant FVIII injection being 48%. Low-dose aspirin was continued concomitantly. The EVAR intervention was carried out under general anaesthesia. Both common femoral arteries were surgically exposed, and heparin (5000 units) was administered intravenously. A Cook (Cook Medical Inc.: Bloomington, Indiana, United States of America) aorto-bi-iliac endograft with intern iliac branch endograft (ZBIS) was successfully deployed with no intraoperative complications. Completion angiography showed stent-graft patency, complete exclusion of the aneurysm and absence of endoleaks. Heparin was reversed with protamine sulphate in a 1:1 ratio (50 mg). The estimated blood loss was 150 mL. Modelling reconstructions of the anatomical structures using CT scans prior to and 3 months after the intervention are shown in Figs. 1 and 2. The first postoperative day was marked by the development of a unilateral haematoma at the femoral artery access, just prior to the third administration of recombinant FVIII concentrate. The patient was thus given 2500 IU of recombinant FVIII concentrate thrice daily for 24 h, followed by 3000 IU twice daily for 48 h. Upon leaving the hospital, the patient was administered 4000 IU of recombinant FVIII concentrate per day for 3 days, followed by a 2-day pause, and then another 3-day course of 4000 IU of recombinant FVIII concentrate once daily. At 9-month follow-up, CT scan results were satisfactory, and no endoleak or bleeding complications were observed. Screening for anti-FVIII inhibitor was negative following the surgery. EVAR and open surgery are both possible for patients when required anatomical conditions are present: aortic neck of minimum 15 mm and no more than 60° angulation with the aortic aneurysm, absence of mural thrombus regarding renal arteries, aortic diameter of minimum 18 mm at its bifurcation and large (minimum 10 mm) and non-tortuous iliac axes. Endovascular treatment options should be considered Haemophilia (2015), 21, e223--e259

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(a)

(b)

Fig. 2. a. Postoperative CT scan 3D reconstruction. b. Bifurcated aortic endograft and iliac branch graft drawing (courtesy of Cook Medical Inc, Bloomington, IN, USA). (CT: coeliac trunk, SMA: superior mesenteric artery, LRA: left renal artery, RRA: right renal artery, SNCS: suprarenal non-covered stent, EMB: endograft main body, RIL: right iliac limb, LIL: left iliac limb, IIB: internal iliac branch, EIB: external iliac branch, IIA: internal iliac artery).

in patients with haemophilia. In our times, the technology makes EVAR also possible in difficult anatomical situation using branched or fenestrated endografts. This indication rests limited to fragile or haemorrhage risk patients. In complicated cases with limited anatomical criteria and important risk of rupture or symptomatic aneurysm, complications to EVAR, as dissection, visceral artery thrombus embolization or covering, colic or spinal cord ischaemia, leg thrombosis, endoleaks or endograft infection may occur. These types of complications vary from 0.5% to 5% in the literature [3]. To our knowledge, five other haemophilic patients treated with open surgery or EVAR were reported in the literature: one with severe haemophilia B, three with, respectively, moderate, mild and severe haemophilia A, and the remaining one with haemophilia B (severity not reported) [4–8]. In patients with normal coagulation patterns, open abdominal aortic aneurysm (AAA) surgical repair usually leads to major intraoperative blood loss, whereas EVAR is associated with a significant reduction in blood loss and transfusion requirement. In our patient, the estimated blood loss was 150 mL, which is similar to the amount reported for the severe haemophilia B patient [4]. This figure must be compared with total blood loss estimated at 1300 and 1700 mL in haemophilia patients following surgical graft replacement [5,7]. A distinctive feature pertaining to our patient was the short duration of hospitalization, namely, 6 days in Haemophilia (2015), 21, e223--e259

our case versus 18, 17 and 20 days in other haemophilia cases [5,7,8]. Another positive issue to focus on is the cost-saving aspect of EVAR, as the amount of recombinant FVIII units to be administered to haemophilia patients during this procedure is significantly less than in standard surgery. In open surgery, it is recommended to maintain FVIII levels at 100% during the first postoperative week, and at 50% during the second, which represents a significantly higher number of FVIII units than in our case. Equally interesting to note is that in our patient, FVIII replacement therapy was twofold less (27 500 IU) than in the other haemophilia cases reported in the literature (50 000 to 60 000) [5–7]. A last point to consider is that in recent years, endovascular iliac artery treatment has become increasingly safe when performed in the general population: a multicentre prospective study involving 39 cases, published in 2013, reported a success rate of 90%, with no death at 30-day follow-up [9]. Because cumulative inhibitor risk increases with age, the development of inhibitors is also potentially problematic for elderly haemophilia patients [10]. According to UK surveillance data, the cumulative risks in mild and moderate haemophilia are 6%, 10% and 12% at 15, 50 and 75 years of age respectively. Older individuals with mild haemophilia A may be at increased risk for inhibitor development when receiving intensive perioperative factor concentrates, and should be monitored for the presence of © 2015 John Wiley & Sons Ltd

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inhibitor development subsequent to factor exposure [10,11]. Our case report clearly shows that EVAR can safely be conducted in haemophilic patients by means of perioperative factor replacement. The choice of this procedure has allowed us to significantly reduce both the haemorrhagic risk associated with the procedure, as well as the cost related to clotting factor replacement therapy. In our opinion, EVAR should be considered as first-line therapy in patients with clinically relevant haemorrhagic disorders. Intraoperative anticoagulant treatment and long-term antiplatelet

References 1 Franchini M, Mannucci PM. Past, present and future of hemophilia: a narrative review. Orphanet J Rare Dis. 2012; 7: 24. 2 Jacquemin M, Vantomme V, Buhot C et al. CD4+ T-Cell clones specific for wild-type factor VIII: a molecular machanism responsible for higher incidence of inhibitor formation in mild/moderate hemophilia A. Blood. 2003;101: 1351–8. 3 Maleux G, Koolen M, Heye S. Complications after endovascular aneurysm repair. Semin Intervent Radiol 2009; 26: 3–9. 4 Marrocco-Trischitta MM, Melissano G, Castellano R et al. Endovascular abdominal aortic aneurysm repair in a patient with

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Acknowledgement The authors gratefully acknowledge Dr. Hasan Catovic from CSL Behring for his assistance in the preparation of the manuscript.

Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.

severe hemophilia B. J Endovasc Ther. 2009; 16: 120–3. Kobayashi M, Matsushita M, Nishikimi N et al. Treatment for abdominal aortic aneurysm in a patient with hemophilia A: a case report and review of the literature. J Vasc Surg 1997; 25: 945–8. Brown B, Steed DL, Webster MW et al. General surgery in adult hemophiliacs. Surgery 1986; 99: 154–9. Bergqvist D, Thimberg L, Bergentz SE et al. Abdominal aortic aneurysm surgery in a hemophiliac. Vasa 1985; 14: 394–7. Gula G, Frezza G. Successful surgery in a patient with haemophilia B: report of case with abdominal aneurysm. Vasc Surg. 1973; 7: 183–7.

9 Maurel B, Bartoli MA, Jean-Baptiste E et al. Perioperative evaluation of iliac ZBIS branch devices: a French multicenter study. Ann Vasc Surg 2013; 2: 131–8. 10 Darby SC, Keeling DM, Spooner RJ et al. The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977-99. J Thromb Haemost 2004; 2: 1047–54. 11 Eckhardt CL, Menke LA, van Ommen CH et al. Intensive peri-operative use of factor VIII and the Arg593–>Cys mutation are risk factors for inhibitor development in mild/moderate hemophilia A. J Thromb Haemost 2009; 7(Suppl 6): 930– 7.

Comment on: Khair K. Compliance, concordance and adherence: what are we talking about? Haemophilia Sept 2014;20(5):601–3. N . A . D U N C A N , C . P . R O B E R S O N and A . D . S H A P I R O Indiana Hemophilia and Thrombosis Center, Inc, Indianapolis, Indiana

“I have been struck again and again by how important measurement is to improving the human condition.” - Bill Gates Kate Khair recently published an interesting commentary proposing a shift from measuring treatment adherence to encouraging concordance, defined in the article as “an agreement between the patient and the provider about how best the patient can manage their treatment.” Khair suggests that if tools designed to Correspondence: Natalie A Duncan, Indiana Hemophilia and Thrombosis Center, Inc, 8326 Naab Road, Indianapolis, IN 46260. Tel.: 317 871 0011; fax: 317 871 0010; e-mail: [email protected] Accepted after revision 16 January 2015 © 2015 John Wiley & Sons Ltd

measure adherence such as the VERITAS-Pro do not actually improve adherence, we should rather focus our efforts on supporting persons with haemophilia (PWH) in their lifelong need to self-administer intravenous therapy. As the authors of the Validated Hemophilia Regimen Treatment Adherence Scales (VERITAS-Pro and VERITAS-PRN) [1,2], we would like to respond to Dr. Khair’s article. We agree and believe that the prevailing approach to care for PWH is in agreement with Khair’s sentiment regarding the involvement of patients in making treatment decisions, individualizing tailored treatment regimens, asking patients for their input on treatment adherence and encouraging age and condition-matched mentorship among patients. Khair offers the specific example of a patient engaged Haemophilia (2015), 21, e223--e259

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Endovascular abdominal aortic aneurysm repair in a mild haemophilia A patient: a case report.

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