Skip to main navigation menu Skip to main content Skip to site footer
##common.pageHeaderLogo.altText##

Systematic review

Vol. 153 No. 8 (2023)

Management of abdominal pseudotumours in haemophilia: a systematic review

  • Franz von Stauffenberg
  • Inga Hegemann
  • Rahel Schwotzer
  • Kuno Lehmann
  • Jeannette Widmer
DOI
https://doi.org/10.57187/smw.2023.40094
Cite this as:
Swiss Med Wkly. 2023;153:40094
Published
15.08.2023

Summary

AIM: Haemophilic pseudotumours are complications in patients with haemophilia A or B and result from locally repetitive bleeding, mainly in the musculoskeletal system. Abdominal haemophilic pseudotumours are exceptionally rare but may cause severe complications. This systematic review aimed to evaluate therapy strategies for symptomatic abdominal haemophilic pseudotumours.

METHODS: We systematically searched three databases (Medline [PubMed], Web of Science and EMBASE) for publications published between 1995 and 2023. Two reviewers independently selected the studies, extracted data and performed a quality assessment using the JBI critical appraisal checklist.

RESULTS: From a total of 1199 articles, 39 articles describing 41 cases were included for final analysis. Conservative or interventional treatment was performed in 12 cases. In eight cases, a step-up to surgical therapy after interventional treatment was indicated. Primary surgical therapy was performed in 21 cases. Failure to cure was documented in 50% (n = 6) of patients treated in the first group, with a mortality rate of 16.6% (n = 2). Interventional therapy with a step-up to surgery showed no morbidity or mortality. Primary surgical resection documented favourable results in 66.6% (n = 14), with failure to cure in 9.5% (n = 2) and a mortality rate of 14.3% (n = 3).

CONCLUSION: Primary surgical resection can be a first-line therapy for symptomatic, abdominal haemophilic pseudotumours, whereas preoperative embolisation could be used as a bridging therapy before surgery, especially in emergency settings. Diagnostic biopsy and percutaneous drainage should be avoided to prevent complications.

References

  1. van den Berg HM, De Groot PH, Fischer K. Phenotypic heterogeneity in severe hemophilia. J Thromb Haemost. 2007 Jul;5 Suppl 1:151–6. 10.1111/j.1538-7836.2007.02503.x DOI: https://doi.org/10.1111/j.1538-7836.2007.02503.x
  2. Hermans C, Dolan G, Jennings I, Windyga J, Lobet S, Rodríguez-Merchán EC, et al. Managing Haemophilia for Life: 5th Haemophilia Global Summit. Eur J Haematol. 2015 Oct;95 Suppl 78:1–25. 10.1111/ejh.12617 DOI: https://doi.org/10.1111/ejh.12617
  3. Castaman G, Matino D. Hemophilia A and B: molecular and clinical similarities and differences. Haematologica. 2019 Sep;104(9):1702–9. 10.3324/haematol.2019.221093 DOI: https://doi.org/10.3324/haematol.2019.221093
  4. Srivastava A, Santagostino E, Dougall A, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia, 2020. 26 Suppl 6: p. 1-158. DOI: https://doi.org/10.1111/hae.14046
  5. Olasupo OO, Lowe MS, Krishan A, Collins P, Iorio A, Matino D. Clotting factor concentrates for preventing bleeding and bleeding-related complications in previously treated individuals with haemophilia A or B. Cochrane Database Syst Rev. 2021 Aug;8(8):CD014201. DOI: https://doi.org/10.1002/14651858.CD014201
  6. Lim MY, Nielsen B, Ma A, Key NS. Clinical features and management of haemophilic pseudotumours: a single US centre experience over a 30-year period. Haemophilia. 2014 Jan;20(1):e58–62. 10.1111/hae.12295 DOI: https://doi.org/10.1111/hae.12295
  7. Doyle AJ, Back DL, Austin S. Characteristics and management of the haemophilia-associated pseudotumours. Haemophilia. 2020 Jan;26(1):33–40. 10.1111/hae.13870 DOI: https://doi.org/10.1111/hae.13870
  8. Gavrel M, Rafowicz A, d’Oiron R, Franchi-Abella S, Lambert T, Adamsbaum C. Imaging features of atypical bleeds in young patients with hemophilia. Diagn Interv Imaging. 2019 Mar;100(3):135–45. 10.1016/j.diii.2018.11.010 DOI: https://doi.org/10.1016/j.diii.2018.11.010
  9. Lin S, Tong K, Wang G, Zhong Z, Cao S, Feng Z. Clinical characteristics and surgical treatment of haemophilic pseudotumor: A retrospective analysis of thirty-four patients. Haemophilia. 2020 Sep;26(5):873–81. 10.1111/hae.14109 DOI: https://doi.org/10.1111/hae.14109
  10. Rodriguez-Merchan EC. Haemophilic cysts (pseudotumours). Haemophilia. 2002 May;8(3):393–401. 10.1046/j.1365-2516.2002.00609.x DOI: https://doi.org/10.1046/j.1365-2516.2002.00609.x
  11. Rodriguez-Merchan EC. Hemophilic Pseudotumors: diagnosis and Management. Arch Bone Jt Surg. 2020 Mar;8(2):121–30.
  12. Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gøtzsche PC, Ioannidis JP, et al. The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration. J Clin Epidemiol. 2009 Oct;62(10):e1–34. 10.1016/j.jclinepi.2009.06.006 DOI: https://doi.org/10.1016/j.jclinepi.2009.06.006
  13. Fischer K, Berntorp E. Targeting factor replacement therapy in severe hemophilia: which level is important? Semin Thromb Hemost. 2015 Nov;41(8):860–3. 10.1055/s-0035-1552562 DOI: https://doi.org/10.1055/s-0035-1552562
  14. Moola S, Munn Z, Tufanaru C, et al. Chapter 7: Systematic reviews of etiology and risk. In: Aromataris E, Munn Z, editors. JBI Manual for Evidence Synthesis. JBI; 2020. DOI: https://doi.org/10.46658/JBIRM-17-06
  15. Magallón M, Monteagudo J, Altisent C, Ibáñez A, Rodríguez-Pérez A, Riba J, et al. Hemophilic pseudotumor: multicenter experience over a 25-year period. Am J Hematol. 1994 Feb;45(2):103–8. 10.1002/ajh.2830450202 DOI: https://doi.org/10.1002/ajh.2830450202
  16. Ahuja SP, Sidonio R Jr, Raj AB, Bertolone SJ, Silverman C, Antekeier DP, et al. Successful combination therapy of a proximal haemophilic pseudotumour with surgery, radiation and embolization in a child with mild haemophilia A. Haemophilia. 2007 Mar;13(2):209–12. 10.1111/j.1365-2516.2006.01425.x DOI: https://doi.org/10.1111/j.1365-2516.2006.01425.x
  17. Al Saadi AS, Al Wadan AH, El Hamarneh SA, Emad ME. Life-threatening biopsy of an iliopsoas pseudotumour in a patient with haemophilia: a case report. J Med Case Rep. 2008 Apr;2(1):135. 10.1186/1752-1947-2-135 DOI: https://doi.org/10.1186/1752-1947-2-135
  18. Bian YY, Wu H, Huang Z, Zhai J, Liu Y, Weng XS. Surgical treatment of a giant iliopsoas haemophilic pseudotumour with adjacent structure compressions: A case report. Haemophilia. 2017 Nov;23(6):e507–12. 10.1111/hae.13299 DOI: https://doi.org/10.1111/hae.13299
  19. Chatterjee S, Mukhopadhyay R. Intra-Abdominal Mesenteric Haemophilic Pseudotumour in an Undiagnosed Case of Haemophilia: a Rare Cause of Intestinal Obstruction. Indian J Surg. 2020;82(6):1284–6. 10.1007/s12262-020-02274-z DOI: https://doi.org/10.1007/s12262-020-02274-z
  20. Dupont MV, Coche EE. CT and MRI Aspects of an Abdominal Hemophilic Pseudotumor. J Belg Soc Radiol. 2015 Dec;99(2):50–2. 10.5334/jbr-btr.887 DOI: https://doi.org/10.5334/jbr-btr.887
  21. Ferreira de Matos C, Claeyssens-Donadel S, Debard A, Piel-Julian ML. A pelvic mass. Rev Med Interne. 2022;43(5):323–4. 10.1016/j.revmed.2022.02.005 DOI: https://doi.org/10.1016/j.revmed.2022.02.005
  22. Fouda AE, Mansour AK, Al-Tonbary YA. Not a true tumour, but a renal pseudotumour: a case report of an 11.5 year old mild haemophilic child. Haemophilia. 2010 Nov;16(6):956–8. 10.1111/j.1365-2516.2010.02334.x DOI: https://doi.org/10.1111/j.1365-2516.2010.02334.x
  23. Frezin J, Marique L, Coubeau L, Hubert C, Lambert C, Hermans C, et al. Successful emergency resection of a massive intra-abdominal hemophilic pseudotumor. World J Gastrointest Surg. 2015 Mar;7(3):43–6. 10.4240/wjgs.v7.i3.43 DOI: https://doi.org/10.4240/wjgs.v7.i3.43
  24. García-Pérez R, Torres-Salmerón G, Sánchez-Bueno F, García-López A, Parrilla-Paricio P. Intraabdominal hemophilic pseudotumor: case report. Rev Esp Enferm Dig. 2010 Apr;102(4):275–80. 10.4321/S1130-01082010000400009 DOI: https://doi.org/10.4321/S1130-01082010000400009
  25. Garge S, Keshava SN, Moses V, Mammen S, Ahmed M, Chiramel GK, et al. Role of endovascular embolization in treatment of acute bleeding complications in haemophilia patients. Br J Radiol. 2016 Aug;89(1064):20151064. 10.1259/bjr.20151064 DOI: https://doi.org/10.1259/bjr.20151064
  26. Goel MK, Juneja D, Jain SK, Chaudhuri SK, Kumar A. An unusual case of pleural-based tumor with life-threatening post-operative complication. Indian J Crit Care Med. 2012 Jan;16(1):48–51. 10.4103/0972-5229.94436 DOI: https://doi.org/10.4103/0972-5229.94436
  27. Gürkan E, Oçal F. Renal haemophilic pseudotumour. Haemophilia. 2005 Sep;11(5):559–60. 10.1111/j.1365-2516.2005.01126.x DOI: https://doi.org/10.1111/j.1365-2516.2005.01126.x
  28. Hahn SY, Hahn SM, Jin SL, Kim HS, Lyu CJ, Lee JG, et al. Huge retroperitoneal complicated pseudotumour in haemophilia B with inhibitor. Haemophilia. 2016 Jan;22(1):e45–7. 10.1111/hae.12799 DOI: https://doi.org/10.1111/hae.12799
  29. Heaton DC, Robertson RW, Rothwell AG. Iliopsoas haemophiliac pseudotumours with bowel fistulation. Haemophilia. 2000 Jan;6(1):41–3. 10.1046/j.1365-2516.2000.00349.x DOI: https://doi.org/10.1046/j.1365-2516.2000.00349.x
  30. Iqbal M, Comp PC, Wu DH. Progression of an untreated pseudotumor. Haemophilia. 2017 Sep;23(5):e464–6. 10.1111/hae.13284 DOI: https://doi.org/10.1111/hae.13284
  31. Kamal AF, Pradana AS, Prabowo Y. Bilateral iliopsoas haemophilic “soft tissue pseudotumours”: A case report. Int J Surg Case Rep. 2015;13:19–23. 10.1016/j.ijscr.2015.05.018 DOI: https://doi.org/10.1016/j.ijscr.2015.05.018
  32. Keller A, Terrier F, Schneider PA, Bianchi S, Howarth N, De Moerloose P. Pelvic haemophilic pseudotumour: management of a patient with high level of inhibitors. Skeletal Radiol. 2002 Sep;31(9):550–3. 10.1007/s00256-002-0518-8 DOI: https://doi.org/10.1007/s00256-002-0518-8
  33. Kilic YA, Dundar SV, Onat D, Akhan O. Iliopsoas hemophilic pseudotumor with bowel fistulization. Bratisl Lek Listy. 2009;110(11):729–31.
  34. Kumar L, Varghese R, Menon RN, Siddharthan N. Perioperative management of a patient with severe Haemophilia B for abdominal pseudotumour Surgery. Indian J Anaesth. 2015 Jul;59(7):461–2. 10.4103/0019-5049.160978 DOI: https://doi.org/10.4103/0019-5049.160978
  35. Kuo CC, Huang CC, Chu TS. Renal haemophilic pseudotumour. Acta Clin Belg. 2009;64(6):555–6. 10.1179/acb.2009.095 DOI: https://doi.org/10.1179/acb.2009.095
  36. Liu S, Zhou X, Song A, Huo Z, Wang Y, Liu Y. Successful treatment of giant retroperitoneal haemophilic pseudotumour. Postgrad Med J. 2019 Aug;95(1126):457. 10.1136/postgradmedj-2019-136719 DOI: https://doi.org/10.1136/postgradmedj-2019-136719
  37. Liu S, Zhou X, Song A, Huo Z, Wang Y, Liu Y. Successful resection of giant abdominal hemophilic pseudotumor: surgical treatment and follow-up outcomes in one single center. Medicine (Baltimore). 2019 Nov;98(46):e17998. 10.1097/MD.0000000000017998 DOI: https://doi.org/10.1097/MD.0000000000017998
  38. López-Gómez J, Contreras JS, Figueroa-Ruiz M, Servín-Torres E, Velázquez-García J, Bevia-Pérez F, et al. Management of the hemophilic pseudotumor of the abdomen: A rare pathological entity. Int J Surg Case Rep. 2014;5(11):789–92. 10.1016/j.ijscr.2014.08.022 DOI: https://doi.org/10.1016/j.ijscr.2014.08.022
  39. Luther A, Mahajan AV, Pandey S. Hemophilic pseudotumor of abdomen: A rare case report. Indian Journal of Vascular and Endovascular Surgery. 2020;7(1):91–3. 10.4103/ijves.ijves_21_19 DOI: https://doi.org/10.4103/ijves.ijves_21_19
  40. Malkan UY, Gunes G, Eliacik E, et al. Giant Hemophilic Pseudotumor of the Iliopsoas: case Report. UHOD Uluslar Hematol Onkol Derg. 2014;24(4):286–8. DOI: https://doi.org/10.4999/uhod.14501
  41. Nachimuthu G, Arockiaraj J, Krishnan V, Sundararaj GD. Hemophilic pseudotumor of the first lumbar vertebra. Indian J Orthop. 2014 Nov;48(6):617–20. 10.4103/0019-5413.144238 DOI: https://doi.org/10.4103/0019-5413.144238
  42. Nguyen H, Nakakura E, Karp S, et al. Management of an enormous hemophilic pseudotumor with arterial embolization followed by surgical evacuation. Haemophilia. 2008;14:61–61.
  43. O’Dowd M, Geoghegan T, Munk PL, McAuley G, Torreggiani WC. Haemophilic pseudotumour presenting with large bowel obstruction. Australas Radiol. 2006 Aug;50(4):386–8. 10.1111/j.1440-1673.2006.01607.x DOI: https://doi.org/10.1111/j.1440-1673.2006.01607.x
  44. Pennekamp PH, Strauss AC, Klein C, Marx A, Goldmann G, Friedrich M, et al. Giant haemophilic pseudotumour of the pelvis: case report and literature review. Haemophilia. 2015 Nov;21(6):e484–6. 10.1111/hae.12752 DOI: https://doi.org/10.1111/hae.12752
  45. Poudyal BS, Shrestha GS. Giant hemophilic pseudotumor eroding the iliac bone. Oxf Med Case Rep. 2021 Mar;2021(3):omab005. 10.1093/omcr/omab005 DOI: https://doi.org/10.1093/omcr/omab005
  46. Sagheer S, Atkins A, McRae S. Successful use of tranexamic acid in the management of haemophilic pseudotumour. Haemophilia. 2016 Jul;22(4):e306–9. 10.1111/hae.12911 DOI: https://doi.org/10.1111/hae.12911
  47. Salaj P, Gurlich R, Svorcová V, Marková M, Cetkovský P. Prophylactic preparation and surgical extirpation of a very large abdominal blood cyst in a severe haemophilia A patient with inhibitors managed by rFVIIa. Haemophilia. 2009 Jan;15(1):380–2. 10.1111/j.1365-2516.2008.01929.x DOI: https://doi.org/10.1111/j.1365-2516.2008.01929.x
  48. Şenol K, Tütüncü T, Yüksek YN, Dağlar Özdemir G, Güney Y, Kama NA. Management of a recurrent massive abdominal haemophilic pseudotumour with adjuvant radiotherapy. Haemophilia. 2015 Jul;21(4):e333–6. 10.1111/hae.12707 DOI: https://doi.org/10.1111/hae.12707
  49. Serban M, Mihailov D, Savescu D, et al., Long-term outcome of an unusual haemophilic pseudotumour. Hamostaseologie, 2012. 32 Suppl 1(Suppl 1): p. S43-4. DOI: https://doi.org/10.1055/s-0037-1619774
  50. Sevilla J, Alvarez MT, Hernández D, Canales M, De Bustos JG, Magallón M, et al. Therapeutic embolization and surgical excision of haemophilic pseudotumour. Haemophilia. 1999 Sep;5(5):360–3. 10.1046/j.1365-2516.1999.00330.x DOI: https://doi.org/10.1046/j.1365-2516.1999.00330.x
  51. Valentino LA, Martinowitz U, Doolas A, Murali P. Surgical excision of a giant pelvic pseudotumour in a patient with haemophilia A. Haemophilia. 2006 Sep;12(5):541–4. 10.1111/j.1365-2516.2006.01318.x DOI: https://doi.org/10.1111/j.1365-2516.2006.01318.x
  52. Ying SH, Chen WM, Wu PK, Chen CF, Liu CL, Chen TH. Pelvic hemophilic pseudotumor presenting as severe sciatic pain in a patient with no history of hemophilic symptoms. J Orthop Sci. 2012 Jul;17(4):490–4. 10.1007/s00776-011-0094-7 DOI: https://doi.org/10.1007/s00776-011-0094-7
  53. Zheng J, Chen K, Liu F, Deng Y, Mao Z, Lv J, et al. Treatment of pelvic haemophilic pseudotumour: A retrospective study. Haemophilia. 2020 Nov;26(6):e308–14. 10.1111/hae.14148 DOI: https://doi.org/10.1111/hae.14148

Most read articles by the same author(s)