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Cardiovascular & Hematological Disorders-Drug Targets

Editor-in-Chief

ISSN (Print): 1871-529X
ISSN (Online): 2212-4063

Review Article

Management of Musculoskeletal Complications in Patients with Hemophilia: Literature Review and Expert Recommendations

Author(s): E. Carlos Rodriguez-Merchan *

Volume 21, Issue 3, 2021

Published on: 27 April, 2021

Page: [162 - 166] Pages: 5

DOI: 10.2174/1871529X21666210427134232

Price: $65

Abstract

In people with hemophilia, hematological prophylaxis during childhood and adolescence could elude the occurrence of musculoskeletal complications (in joints and muscles) if the concentration of the defective factor is averted from decreasing under 1% of normal. Prompt management is of capital significance as the juvenile skeleton is hypersensitive to the adverse events of the disease; intense structural defects might appear rapidly. Important, articular bleeds and inveterate hypertrophy of the articular synovial membrane must be treated vigorously to preclude joint degeneration (hemophilic arthropathy). At the moment that extreme joint disease is in place with intense affliction, the goal must be to reestablish activity whilst at the same time reducing the peril to the patient. Arthroscopic articular debridement is an efficacious surgical technique to accomplish this goal, particularly around the knee or ankle, and maybe contemplated to be a backup to ankle arthrodesis or ankle or knee replacement in patients of younger age. Eventually, joint replacement can commonly reestablish both articular mobility and function in an unhealthy articulation.

Keywords: Hemophilia, joints, muscles, complications, synovitis, arthropathy, pseudotumors.

Graphical Abstract
[1]
Mahlangu, J.N.; Blanchette, V.; Klamroth, R. Redefining prophylaxis in the modern era. Haemophilia, 2021, 27(3), 21-27.
[http://dx.doi.org/10.1111/hae.14035] [PMID: 32469108]
[2]
Valentino, L.A.; Khair, K. Prophylaxis for hemophilia A without inhibitors: treatment options and considerations. Expert Rev. Hematol., 2020, 13(7), 731-743.
[http://dx.doi.org/10.1080/17474086.2020.1775576] [PMID: 32573295]
[3]
Mancuso, M.E.; Oldenburg, J.; Boggio, L.; Kenet, G.; Chan, A.; Altisent, C.; Seifert, W.; Santagostino, E. High adherence to prophylaxis regimens in haemophilia B patients receiving rIX-FP: Evidence from clinical trials and real-world practice. Haemophilia, 2020, 26(4), 637-642.
[http://dx.doi.org/10.1111/hae.14018] [PMID: 32542961]
[4]
Srivastava, A.; Santagostino, E.; Dougall, A.; Kitchen, S.; Sutherland, M.; Pipe, S.W. WFH guidelines for the management of hemophilia. Haemophilia, 3rd edition; , 2020, 26, pp. (6)1-158.
[5]
van den Berg, H.M.; Mancuso, M.E.; Königs, C.; D’Oiron, R.; Platokouki, H.; Mikkelsen, T.S.; Motwani, J.; Nolan, B.; Santagostino, E. European Pediatric Network for Haemophilia Management (PedNet). ITI Treatment is not first-choice treatment in children with hemophilia A and low-responding inhibitors: Evidence from a PedNet study. Thromb. Haemost., 2020, 120(8), 1166-1172.
[http://dx.doi.org/10.1055/s-0040-1713097] [PMID: 32572865]
[6]
Rodriguez-Merchan, E.C.; Jimenez-Yuste, V.; Aznar, J.A.; Hedner, U.; Knobe, K.; Lee, C.A.; Ljung, R.; Querol, F.; Santagostino, E.; Valentino, L.A.; Caffarini, A. Joint protection in haemophilia. Haemophilia, 2011, 17(2), 1-23.
[http://dx.doi.org/10.1111/j.1365-2516.2011.02615.x] [PMID: 21819491]
[7]
Rodriguez-Merchan, E.C. Musculo-skeletal manifestations of haemophilia. Blood Rev., 2016, 30(5), 401-409.
[http://dx.doi.org/10.1016/j.blre.2016.04.008] [PMID: 27166435]
[8]
Liddle, A.D.; Rodriguez-Merchan, E.C. Evidence-based management of the knee in hemophilia. JBJS Rev., 2017, 5(8), e12.
[http://dx.doi.org/10.2106/JBJS.RVW.16.00100] [PMID: 28832348]
[9]
Rodriguez-Merchan, E.C. Risks and patient outcomes of surgical intervention for hemophilic arthropathy. Expert Rev. Hematol., 2019, 12(5), 325-333.
[http://dx.doi.org/10.1080/17474086.2019.1602035] [PMID: 30929534]
[10]
Rodríguez-Merchán, E.C. The role of orthopaedic surgery in haemophilia: current rationale, indications and results. EFORT Open Rev., 2019, 4(5), 165-173.
[http://dx.doi.org/10.1302/2058-5241.4.180090] [PMID: 31191984]
[11]
Escobar, M.A.; Brewer, A.; Caviglia, H.; Forsyth, A.; Jimenez-Yuste, V.; Laudenbach, L.; Lobet, S.; McLaughlin, P.; Oyesiku, J.O.O.; Rodriguez-Merchan, E.C.; Shapiro, A.; Solimeno, L.P. Recommendations on multidisciplinary management of elective surgery in people with haemophilia. Haemophilia, 2018, 24(5), 693-702.
[http://dx.doi.org/10.1111/hae.13549] [PMID: 29944195]
[12]
Rodríguez-Merchán, E.C. Effects of hemophilia on articulations of children and adults. Clin. Orthop. Relat. Res., 1996, (328), 7-13.
[http://dx.doi.org/10.1097/00003086-199607000-00003] [PMID: 8653981]
[13]
Rodriguez-Merchan, E.C. Articular bleeding in hemophilia. Cardiovasc. Hematol. Disord. Drug Targets, 2016, 16(1), 21-24.
[http://dx.doi.org/10.2174/1871529X16666160613114506] [PMID: 28049407]
[14]
De la Corte-Rodriguez, H.; Rodriguez-Merchan, E.C.; Alvarez-Roman, M.T.; Martin-Salces, M.; Romero-Garrido, J.A.; Jimenez-Yuste, V. Accelerating recovery from acute hemarthrosis in patients with hemophilia: the role of joint aspiration. Blood Coagul. Fibrinolysis, 2019, 30(3), 111-119.
[http://dx.doi.org/10.1097/MBC.0000000000000803] [PMID: 30958454]
[15]
De la Corte-Rodriguez, H.; Rodriguez-Merchan, E.C.; Jimenez-Yuste, V. Point-of-care ultrasonography in orthopedic management of hemophilia: Multiple uses of an effective tool. HSS J., 2018, 14(3), 307-313.
[http://dx.doi.org/10.1007/s11420-018-9604-x] [PMID: 30258338]
[16]
De la Corte-Rodriguez, H.; Rodriguez-Merchan, E.C.; Alvarez-Roman, M.T.; Martin-Salces, M.; Martinoli, C.; Jimenez-Yuste, V. The value of HEAD-US system in detecting subclinical abnormalities in joints of patients with hemophilia. Expert Rev. Hematol., 2018, 11(3), 253-261.
[http://dx.doi.org/10.1080/17474086.2018.1435269] [PMID: 29383965]
[17]
Rodriguez-Merchan, E.C.; Goddard, N.J.; Lee, C.A. Musculoskeletal Aspects of Haemophilia; Blackwell Science Ltd: Oxford, UK, 2000.
[18]
Rodriguez-Merchan, E.C. Management of orthopaedic complications of haemophilia. J Bone Joint Surg Br, 1998, 80B, 191-196.
[PMID: 9546442]
[19]
Tolend, M.; Majeed, H.; Soliman, M.; Daruge, P.; Bordalo-Rodrigues, M.; Dertkigil, S.S.J.; Gibikote, S.; Keshava, S.N.; Stimec, J.; Dunn, A.; Li, Y.J.; Blanchette, V.; Lundin, B.; Doria, A.S. Critical appraisal of the International Prophylaxis Study Group magnetic resonance image scale for evaluating haemophilic arthropathy. Haemophilia, 2020, 26(4), 565-574.
[http://dx.doi.org/10.1111/hae.14032] [PMID: 32497355]
[20]
Soliman, M.; Daruge, P.; Dertkigil, S.S.J.; De Avila Fernandes, E.; Negrao, J.R.; de Aguiar Vilela Mitraud, S.; Sakuma, E.T.I.; Fernandes, A.R.C.; Zhang, N.; Huo, A.; Li, Y.J.; Zhou, F.; Rodrigues, B.M.; Mohanta, A.; Blanchette, V.S.; Doria, A.S. Imaging of haemophilic arthropathy in growing joints: pitfalls in ultrasound and MRI. Haemophilia, 2017, 23(5), 660-672.
[http://dx.doi.org/10.1111/hae.13249] [PMID: 28574216]
[21]
Rodriguez-Merchan, E.C.; Valentino, L.A. Safety of radiation exposure after radiosynovectomy in paediatric patients with haemophilia. Haemophilia, 2015, 21(4), 411-418.
[http://dx.doi.org/10.1111/hae.12668] [PMID: 25854422]
[22]
Rodriguez-Merchan, E.C. Radiosynovectomy in haemophilia. Blood Rev., 2019, 35, 1-6.
[http://dx.doi.org/10.1016/j.blre.2019.01.002] [PMID: 30704767]
[23]
Fernandez-Palazzi, F.; Rivas, S.; Cibeira, J.L.; Dib, O.; Viso, R. Radioactive synoviorthesis in hemophilic hemarthrosis: materials, techniques, and dangers. Clin. Orthop. Relat. Res., 1996, (328), 14-18.
[http://dx.doi.org/10.1097/00003086-199607000-00004] [PMID: 8653947]
[24]
Rivard, G.E.; Girard, M.; Bélanger, R.; Jutras, M.; Guay, J.P.; Marton, D. Synoviorthesis with colloidal 32P chromic phosphate for the treatment of hemophilic arthropathy. J. Bone Joint Surg. Am., 1994, 76(4), 482-488.
[http://dx.doi.org/10.2106/00004623-199404000-00002] [PMID: 8150815]
[25]
Smith, M.A.; Urquhart, D.R.; Savidge, G.F. The surgical management of varus deformity in haemophilic arthropathy of the knee. J. Bone Joint Surg. Br., 1981, 63-B(2), 261-265.
[http://dx.doi.org/10.1302/0301-620X.63B2.7217153] [PMID: 7217153]
[26]
Pearce, M.S.; Smith, M.A.; Savidge, G.F. Supramalleolar tibial osteotomy for haemophilic arthropathy of the ankle. J. Bone Joint Surg. Br., 1994, 76(6), 947-950.
[http://dx.doi.org/10.1302/0301-620X.76B6.7983125] [PMID: 7983125]
[27]
Martinowitz, U.; Schulman, S.; Horoszowski, H.; Heim, M. Role of fibrin sealants in surgical procedures on patients with hemostatic disorders. Clin. Orthop. Relat. Res., 1996, (328), 65-75.
[http://dx.doi.org/10.1097/00003086-199607000-00013] [PMID: 8653980]
[28]
Ribbans, W.J.; Phillips, A.M. Hemophilic ankle arthropathy. Clin. Orthop. Relat. Res., 1996, (328), 39-45.
[http://dx.doi.org/10.1097/00003086-199607000-00009] [PMID: 8653975]
[29]
Rodriguez-Merchan, E.C. Management of hemophilic arthropathy of the ankle. Cardiovasc. Hematol. Disord. Drug Targets, 2017, 17(2), 111-118.
[http://dx.doi.org/10.2174/1871529X17666170908110758] [PMID: 28891453]
[30]
Horoszowski, H.; Heim, M.; Schulman, S.; Varon, D.; Martinowitz, U. Multiple joint procedures in a single operative session on hemophilic patients. Clin. Orthop. Relat. Res., 1996, (328), 60-64.
[http://dx.doi.org/10.1097/00003086-199607000-00012] [PMID: 8653979]
[31]
Kelley, S.S.; Lachiewicz, P.F.; Gilbert, M.S.; Bolander, M.E.; Jankiewicz, J.J. Hip arthroplasty in hemophilic arthropathy. J. Bone Joint Surg. Am., 1995, 77(6), 828-834.
[http://dx.doi.org/10.2106/00004623-199506000-00003] [PMID: 7782355]
[32]
Löfqvist, T.; Sanzén, L.; Petersson, C.; Nilsson, I.M. Total hip replacement in patients with hemophilia. 13 hips in 11 patients followed for 1-16 years. Acta Orthop. Scand., 1996, 67(4), 321-324.
[http://dx.doi.org/10.3109/17453679609002323] [PMID: 8792731]
[33]
Rodriguez-Merchan, E.C. Complications of muscle hematomas in hemophilia. Cardiovasc. Hematol. Disord. Drug Targets, 2020, 20(4), 242-248.
[http://dx.doi.org/10.2174/1871529X20666200415121409] [PMID: 32294050]

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