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Deep vein and intracardiac thrombosis during the post-partum period in Behçet’s disease

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Abstract

A 22-year-old woman presented on the 10th post-partum day with deep vein thrombosis involving the right ilio-femoral and popliteal veins. This thrombosis was refractory to conventional anticoagulation and subsequently over a period of 6 weeks progressed to involve inferior vena cava and right ventricle. A diagnosis of Behçet’s disease was made on the clinical grounds of fever, night sweats, and recurrent oral ulcers. In view of refractory thrombosis, anticoagulation with lepirudin was commenced followed by thrombolysis with streptokinase. After thrombolysis, anticoagulation was switched to fondaparinux. Intracardiac thrombus, oral ulcers, constitutional symptoms, and inflammatory indices resolved on 20 mg of oral prednisolone. This case highlights that management of thrombosis unresponsive to conventional anticoagulation requires careful consideration of the underlying conditions, the sites of thrombosis, as well as of the available treatment options. Intravenous lepirudin is a valuable therapeutic option when anticoagulation with warfarin or LMWH is not efficacious. Thrombolytic therapy may be used to treat intracardiac thrombi more rapidly, and possibly more efficiently, than surgery. The addition of corticosteroids or other immunosuppressive drugs is necessary in order to achieve a full resolution of thrombosis.

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References

  1. Heit JA. Venous thromboembolism: disease burden, outcomes and risk factors. J Thromb Haemost. 2005;3:1611–7.

    Article  CAS  PubMed  Google Scholar 

  2. Pomp ER, Lenselink AM, Rosendaal FR, Doggen CJ. Pregnancy, the postpartum period and prothrombotic defects: risk of venous thrombosis in the MEGA study. J Thromb Haemost. 2008;6:632–7.

    Article  CAS  PubMed  Google Scholar 

  3. McColl MD, Walker ID, Greer IA. Risk factors for venous thromboembolism in pregnancy. Curr Opin Pulm Med. 1999;5:227–32.

    Article  CAS  PubMed  Google Scholar 

  4. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med. 1999;341:1284–91.

    Article  CAS  PubMed  Google Scholar 

  5. Leiba M, Sidi Y, Gur H, Leiba A, Ehrenfeld M. Behçet’s disease and thrombophilia. Ann Rheum Dis. 2001;60:1081–5.

    Article  CAS  PubMed  Google Scholar 

  6. Sarica-Kucukoglu R, Akdag-Kose A, Kayabal IM, Yazganoglu KD, Disci R, Erzengin D, et al. Vascular involvement in Behçet’s disease: a retrospective analysis of 2319 cases. Int J Dermatol. 2006;45:919–21.

    Article  PubMed  Google Scholar 

  7. Uzun S, Alpsoy E, Durdu M, Akman A. The clinical course of Behçet’s disease in pregnancy: a retrospective analysis and review of the literature. J Dermatol. 2003;30:499–502.

    PubMed  Google Scholar 

  8. Barkhausen J, Hunold P, Eggebrecht H, Schuler WO, Sabin GV, Erbel R, et al. Detection and characterization of intracardiac thrombi on MR imaging. AJR Am J Roentgenol. 2002;179:1539–44.

    PubMed  Google Scholar 

  9. Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behçet’s disease: a systematic review. Chest. 2000;118:479–87.

    Article  CAS  PubMed  Google Scholar 

  10. Başak M, Gül S, Yazgan Y, Cankir Z, Danaci M, Demirtürk L, et al. A case of rapidly progressive pulmonary aneurysm as a rare complication of Behçet’s syndrome—a case report. Angiology. 1998;49(5):403–8.

    Article  PubMed  Google Scholar 

  11. Yasuo M, Nagano S, Yazaki Y, Koizumi T, Kitabayashi H, Imamura H, et al. Pulmonary embolism due to right ventricular thrombus in a case of Behçet’s disease. Jpn Circ J. 1999;63:909–11.

    Article  CAS  PubMed  Google Scholar 

  12. Basaran Y, Degertekin M, Direskeneli H, Yakut C. Cardiac thrombosis in a patient with Behçet’s disease: two years follow-up. Int J Card Imaging. 2000;16:377–82.

    Article  CAS  PubMed  Google Scholar 

  13. Gurgun C, Sagcan A, Cinar CS, Yagdi T, Zoghi M, Tekten T, et al. Right atrial and ventricular thrombi in Behçet’s disease: a case report and review of literature. Blood Coagul Fibrinolysis. 2000;11:107–10.

    CAS  PubMed  Google Scholar 

  14. Dincer I, Dandachi R, Atmaca Y, Erol C, Caglar N, Oral D. A recurrent right heart thrombus in a patient with Behçet’s disease. Echocardiography. 2001;18(1):15–8.

    Article  CAS  PubMed  Google Scholar 

  15. Ozatli D, Kav T, Haznedaroglu IC, Büyükaşik Y, Koşar A, Ozcebe O, et al. Cardiac and great vessel thrombosis in Behçet’s disease. Intern Med. 2001;40(1):68–72.

    Article  CAS  PubMed  Google Scholar 

  16. Baykan M, Celik S, Erdol C, Baykan EC, Durmus I, Bahadir S, et al. Behçet’s disease with a large intracardiac thrombus: a case report. Heart. 2001;85:E7.

    Article  CAS  PubMed  Google Scholar 

  17. Houman M, Ksontini I, Ben Ghorbel I, Lamloum M, Braham A, Mnif E, et al. Association of right heart thrombosis, endomyocardial fibrosis, and pulmonary artery aneurysm in Behçet’s disease. Eur J Intern Med. 2002;13:455.

    Article  PubMed  Google Scholar 

  18. Altunkeser BB, Gormus N, Ozdemir K, Durgut K, Ozdemir A, Solak H, et al. A recurrent right atrial thrombus: a suggestive sign for Behçet’s disease? A case report. Heart Vessels. 2002;16:196–7.

    Article  PubMed  Google Scholar 

  19. Goktekin O, Korkmaz C, Timuralp B, Kudaiberdieva G, Gorenek B, Cavusoglu Y, et al. Widespread thrombosis associated with recurrent intracardiac masses in a patient with Behçet’s disease. Int J Cardiovasc Imaging. 2002;18:431–4.

    Article  PubMed  Google Scholar 

  20. Kaya A, Ertan C, Gurkan OU, Fitoz S, Atasoy C, Kilickap M, et al. Behçet’s disease with right ventricle thrombus and bilateral pulmonary artery aneurysms—a case report. Angiology. 2004;55:573–5.

    Article  PubMed  Google Scholar 

  21. Kaneko Y, Tanaka K, Yoshizawa A, Yasuoka H, Suwa A, Satoh T, et al. Successful treatment of recurrent intracardiac thrombus in Behçet’s disease with immunosuppressive therapy. Clin Exp Rheumatol. 2005;23:885–7.

    CAS  PubMed  Google Scholar 

  22. Ozer N, Ciftci O, Demirci M, Atalar E, Ovunc K, Aksoyek S, et al. Right ventricular outflow tract thrombus in a case of Behçet’s disease. J Am Soc Echocardiogr. 2005;18:380–2.

    Article  PubMed  Google Scholar 

  23. Vahedian J, Sadeghpour A. Arterial homograft and medical therapy in pseudoaneurysm of infrarenal aorta concomitant with recurrent right ventricular thrombus in Behçet’s disease. Saudi Med J. 2006;27:1401–3.

    PubMed  Google Scholar 

  24. Ernam D, Atalay F, Alp A, Hasanoğlu HC. A Behçet’s disease patient with intracardiac thrombus, pulmonary artery aneurysms complicating recurrent pulmonary thromboembolism. Tuberk Toraks. 2006;54(2):168–71.

    PubMed  Google Scholar 

  25. Dogan SM, Birdane A, Korkmaz C, Ata N, Timuralp B. Right ventricular thrombus with Behçet’s syndrome: successful treatment with warfarin and immunosuppressive agents. Tex Heart Inst J. 2007;34:360–2.

    PubMed  Google Scholar 

  26. Chiari E, Fracassi F, D’Aloia A, Vizzardi E, Zanini G, Rocca P, et al. Right ventricular thrombus and pulmonary thromboembolism/thrombosis in Behçet’s disease: a case report. J Am Soc Echocardiogr. 2008;21:1079.

    PubMed  Google Scholar 

  27. Chang JE, Lee YH, Lee J. Multiple cardiovascular complications in a patient with Behçet’s disease. Korean J Intern Med. 2008;23:100–2.

    Article  PubMed  Google Scholar 

  28. El Houari T, Oukerraj L, Ghzaiel L, Fellat I, Azeroual M, Serraj K, et al. Management of Behçet disease with multiple complications. Hellenic J Cardiol. 2009;50(5):420–2.

    PubMed  Google Scholar 

  29. Krause I, Weinberger A. Vasculo-Behçet’s disease. Isr Med Assoc J. 2002;4:636–7.

    PubMed  Google Scholar 

  30. Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS. Treatment of venous thrombosis associated with Behçet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol. 2008;27:201–5.

    Article  PubMed  Google Scholar 

  31. Chartier L, Bera J, Delomez M, Asseman P, Beregi JP, Bauchart JJ, et al. Free-floating thrombi in the right heart: diagnosis, management, and prognostic indexes in 38 consecutive patients. Circulation. 1999;99:2779–83.

    CAS  PubMed  Google Scholar 

  32. Mendes LA, Magraw LL, Aldea GS, Davidoff R. Right ventricular thrombus: an unusual manifestation of Behçet’s disease. J Am Soc Echocardiogr. 1994;7:438–40.

    CAS  PubMed  Google Scholar 

  33. Kirali K, Civelek A, Daglar B, Sismanoglu M, Akinci E, Berki T, et al. An uncommon complication of Behçet’s disease: intracardiac thrombosis needing surgical treatment. Thorac Cardiovasc Surg. 1998;46:102–5.

    Article  CAS  PubMed  Google Scholar 

  34. Duchene F, Berthier S, de Wazieres B, Zyka F, Leroy J, Estavoyer JM et al. Behçet’s disease with cardiac and pulmonary manifestations. Presse Med 1998;27:1674–6.

    Google Scholar 

  35. Harmouche H, Tazi Mezalek Z, Adnaoui M, Aouni M, Mohattane A, Maaouni A et al. Association aneurysme de l’artere pulmonaire, thromboses intracardiaques et anticorps antiphospholipides au cours de la maladie de Behcet: a propo d’un cas. Rev Med Interne 1998;19:512–5.

    Google Scholar 

  36. Roguin A, Edoute Y, Milo S, Shtiwi S, Markiewicz W, Reisner SA. A fatal case of Behçet’s disease associated with multiple cardiovascular lesions. Int J Cardiol 1997;59:267–3.

    Google Scholar 

  37. Huong DL, Wechsler B, Papo T, de Zuttere D, Bletry O, Hernigou A et al. Endomyocardial fibrosis in Behçet’s disease. Ann Rheum Dis 1997;56:205–8.

    Google Scholar 

  38. Huong DL, Dolmazon C, De Zuttere D, Wechsler B, Godeau P, Piette JC. Complete recovery of right intraventricular thrombus and pulmonary arteritis in Behçet’s disease. Br J Rheumatol 1997;36:130–2.

    Google Scholar 

  39. Soulami S, Nour-Eddine M, Azzouzi L, Bennis A, Chraibi N. Fibrose endomyocardique du coeur droit au cours de la maladie de Behçet. Arch Mal Coeur Vaiss 1996;89:917–21.

    Google Scholar 

  40. Nakata Y, Awazu M, Kojima Y, Tokumura M, Yamagishi H, Yamashita N. Behçet’s disease presenting with a right atrial vegetation. Pediatr Cardiol 1995;16:150–2.

    Google Scholar 

  41. Islim IF, Gill MD, Situnayake D, Watson RD. Successful treatment of right atrial thrombus in a patient with Behçet’s disease. Ann Rheum Dis 1994;53:550–1.

    Google Scholar 

  42. Madanat WY, Prokaeva TB, Kotel’nikova GP, Alekberova ZS. Endocarditis with left atrial thrombus formation in Behçet’s disease mimicking myxoma. J Rheumatol 1993;20:1982–4.

    Google Scholar 

  43. Sayin AG, Vural FS, Bozkurt AK, Oz B, Uygun N. Right atrial thrombus mimicking myxoma and bilateral pulmonary artery aneurysms in a patient with Behçet’s disease: a case report. Angiology 1993;44:915–8.

    Google Scholar 

  44. Koc Y, Gullu I, Akpek G, Akpolat T, Kansu E, Kiraz S et al. Vascular involvement in Behçet’s disease. J Rheumatol 1992;19:402–10.

    Google Scholar 

  45. Pottiez Y, Francois D. Thrombus intraventriculaire (iatrogene?) et maladie de Behçet. Rev Rhum 1991;58:543–4.

    Google Scholar 

  46. El-Ramahi KM, Fawzy ME, Sieck JO, Vanhaleweyk G. Cardiac and pulmonary involvement in Behçet’s disease. Scand J Rheumatol 1991;20:373–6.

    Google Scholar 

  47. Vanhaleweyk G, El-Ramahi KM, Hazmi M, Sieck JO, Zaman L, Fawzy M. Right atrial, right ventricular and left ventricular thrombi in (incomplete) Behçet’s disease. Eur Heart J 1990;11:957–9.

    Google Scholar 

  48. Lie JT. Cardiac and pulmonary manifestations of Behçet syndrome. Pathol Res Pract 1988;183:347–52.

    Google Scholar 

  49. Augarten A, Apter S, Theodor R. Right ventricular thrombus and pulmonary arteritis in Behçet’s disease. Isr J Med Sci 1987;23:900–1.

    Google Scholar 

  50. Candan I, Erol C, Sonel A, Akalin H. Behçet’s disease: cardiac and pulmonary involvement. Eur Heart J 1986;7:999–1002.

    Google Scholar 

  51. Buge A, Escourolle R, Chomette G, Rancurel G, Dechy H, Hauw JJ et al. Maladie de Behçet avec manifestations neurologiques et fibrose endocardique du coeur droit: etude anatomoclinique d’une observation. Ann Med Interne (Paris) 1977;128:411–9.

    Google Scholar 

  52. Davies JD. Behçet’s syndrome with haemoptysis and pulmonary lesions. J Pathol 1973;109:351–6.

    Google Scholar 

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Correspondence to Prashant Hiwarkar.

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Hiwarkar, P., Stasi, R., Sutherland, G. et al. Deep vein and intracardiac thrombosis during the post-partum period in Behçet’s disease. Int J Hematol 91, 679–686 (2010). https://doi.org/10.1007/s12185-010-0538-4

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  • DOI: https://doi.org/10.1007/s12185-010-0538-4

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