Management of Haemophilic Pseudotumor of Left-Hand in A Paediatric Patient: a Case Report
Main Article Content
Hemophilia is a recessively inherited genetic disorder that causes abnormalities in the blood clotting process, which can lead to bleeding that is difficult to stop. One of the rare but significant complications of hemophilia is hemophilia pseudotumor, which occurs in 1-2% of hemophilia A and B patients. This study aims to analyze and evaluate the management of pseudotumor hemophilia. This study aims to analyze and evaluate the management of hemophilia pseudotumor in pediatric patients, focusing on the case of an 8-year-old boy who developed a pseudotumor in the left hand area due to hemophilia A. This case involves a boy with a history of hemophilia A who developed swelling of the left hand after experiencing trauma. Diagnosis was done through clinical examination, radiology (X-ray and MRI), and hematology evaluation. Treatment was factor VIII therapy, tranexamate, and amputation, as well as rFVIIa for bleeding prevention. The patient showed improved function after left hand amputation. Although conservative management can be considered for newly formed pseudotumors, in this case, a surgical approach was necessary due to the significant size and complications. Hemophilic pseudotumors can cause damage to surrounding tissues and require a multidisciplinary approach for management. Hemophilic pseudotumor is a rare but serious complication in people with hemophilia. Appropriate management, including surgical intervention, can improve the functional outcome of patients, despite the significant risks involved. Further research is needed to develop more effective treatment protocols.
Bocklage, T. J., Quinn, R., Schmit, B., & Verschraegen, C. (2014). Bone and soft tissue tumors: a multidisciplinary review with case presentations.
Doyle, A. J., Back, D. L., & Austin, S. (2020). Characteristics and management of the haemophilia?associated pseudotumours. Haemophilia, 26(1), 33–40. 10.1111/hae.13870
Favaloro, E. J., & Lippi, G. (2015). Laboratory Testing in the era of Direct or non–vitamin K antagonist oral anticoagulants: a practical guide to measuring their activity and avoiding diagnostic errors. Seminars in Thrombosis and Hemostasis, 41(02), 208–227. 10.1055/s-0035-1546827
Hermans, C., Noone, D., Benson, G., Dolan, G., Eichler, H., Jiménez-Yuste, V., Königs, C., Lobet, S., Pollard, D., & Zupan?i?-Šalek, S. (2022). Hemophilia treatment in 2021: Choosing the” optimal” treatment using an integrative, patient-oriented approach to shared decision-making between patients and clinicians. Blood Reviews, 52, 100890. 10.1016/j.blre.2021.100890
Hotea, I., Brinza, M., Blag, C., Zimta, A.-A., Dirzu, N., Burzo, C., Rus, I., Apostu, D., Benea, H., & Marian, M. (2021). Current therapeutic approaches in the management of hemophilia—a consensus view by the Romanian Society of Hematology. Annals of Translational Medicine, 9(13). 10.21037/atm-21-747
Lester, W., Bent, C., Alikhan, R., Roberts, L., Gordon?Walker, T., Trenfield, S., White, R., Forde, C., Arachchillage, D. J., & Committee, B. S. H. (2024). A British Society for Haematology guideline on the assessment and management of bleeding risk prior to invasive procedures. British Journal of Haematology.
Lobet, S., Hermans, C., & Lambert, C. (2014). Optimal management of hemophilic arthropathy and hematomas. Journal of Blood Medicine, 207–218.
Reigstad, O. (2014). Wrist arthroplasty: bone fixation, clinical development and mid to long term results. Acta Orthopaedica, 85(sup354), 1–53. 10.3109/17453674.2014.900597
Scharf, R. E. (2021). Thrombocytopenia and hemostatic changes in acute and chronic liver disease: pathophysiology, clinical and laboratory features, and management. Journal of Clinical Medicine, 10(7), 1530. 10.3390/jcm10071530
Shaw, M., Pelecanos, A. M., & Mudge, A. M. (2020). Evaluation of internal medicine physician or multidisciplinary team comanagement of surgical patients and clinical outcomes: a systematic review and meta-analysis. JAMA Network Open, 3(5), e204088–e204088. 10.1001/jamanetworkopen.2020.4088
Tong, T. K., & Sathar, J. (2018). Haemophilic Pseudotumor: Two Case Reports. Med Rep Case Stud 3: 162. doi: 10.4172/2572-5130.1000162 Page 2 of 4 Med Rep Case Stud, an open access journal ISSN: 2572-5130 Volume 3• Issue 3• 1000162. Figure, 5, 3. 10.4172/2572-5130.1000162
von Kodolitsch, Y., Rybczynski, M., Vogler, M., Mir, T. S., Schüler, H., Kutsche, K., Rosenberger, G., Detter, C., Bernhardt, A. M., & Larena-Avellaneda, A. (2016). The role of the multidisciplinary health care team in the management of patients with Marfan syndrome. Journal of Multidisciplinary Healthcare, 587–614.
Yao, Y., Gao, Q., Li, J., Xue, C., Fang, W., & Jing, J. (2022). Outcome of surgical Management of Hemophilic Pseudotumor: review of 10 cases from single?center. Orthopaedic Surgery, 14(1), 27–34. 10.1111/os.13174
Yao, Y., Gao, Q., Li, J., Xue, C., Fang, W., Jing, J., Rodriguez-Merchan, E. C., Doyle, A. J., Back, D. L., & Austin, S. (2020). Hemophilic pseudotumors: diagnosis and management. Archives of Bone and Joint Surgery, 8(1), 33–40. 10.22038/abjs.2019.40547.2090
Zhai, J., Weng, X., Zhang, B., Peng, H., Bian, Y., & Zhou, L. (2015). Surgical management of hemophilic pseudotumor complicated by destructive osteoarthropathy. Blood Coagulation & Fibrinolysis, 26(4), 373–377. 10.1097/MBC.0000000000000260