Skip to main content
Log in

Paroxysmal nocturnal hemoglobinuria: complete resolution of an occluding inferior vena caval thrombus

  • Published:
Abdominal Imaging Aims and scope Submit manuscript

Abstract

Paroxysmal nocturnal hemoglobinuria is a rare acquired autoimmune disease, and is frequently associated with venous thrombosis. A patient who developed thrombotic occlusion of the inferior vena cava is described. Treatment with heparin and urokinase, followed by oral anticoagulant, was effective in resolving abdominal symptoms. The venous thrombosis resolved completely, but the patient died during treatment of aplastic anemia.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1.
Fig. 2.

Similar content being viewed by others

References

  1. Packman CH (1998) Pathogenesis and management of paroxysmal nocturnal hemoglobinuria. Blood Rev 12:1–11

    Article  PubMed  CAS  Google Scholar 

  2. Chrobak L (2000) Paroxysmal nocturnal hemoglobinuria (membrane defect, pathogenesis, aplastic anemia, diagnosis). ACTA Medica 43:3–9

    PubMed  CAS  Google Scholar 

  3. Ray JG, Burows RF, Ginberg JS, et al. (2000) Paroxysmal nocturnal hemoglobinuria and the risk of venous thrombosis: review and recommendations for management of the pregnant and nonpregnant patient. Haemostasis 30:103–117

    Article  PubMed  CAS  Google Scholar 

  4. Moyo VM, Mukhina GL, Garrett ES, et al. (2004) Natural history of paroxysmal nocturnal hemoglobinuria using modern diagnostic assays. Br J Haematol 126:133–138

    Article  PubMed  CAS  Google Scholar 

  5. Rosse WF, Nishimura J (2003) Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems. Int J Hematol 77:113–120

    Article  PubMed  Google Scholar 

  6. Yamashina M, Ueda E, Kinoshita T, et al. (1990) Inherited complete deficiency of 20-kilodalton homologous restriction factor (CD59) as a cause of paroxysmal nocturnal hemoglobinuria. New Engl J Med 323:1184–1189

    Article  PubMed  CAS  Google Scholar 

  7. Dacie JV, Lewis SM (1972) Paroxysmal nocturnal haemoglobinuria: clinical manifestations, haematology, and nature of the disease. Ser Haemat 3:3–23

    Google Scholar 

  8. Peytremann R, Rhodes RS, Hartmann RC (1972) Thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) with particular reference to progressive, diffuse hepatic venous thrombosis. Ser Haemat 3:115–136

    Google Scholar 

  9. Valla D, Dhumeax D, Babany G, et al. (1987) Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. Gastroenterology 93:569–575

    PubMed  CAS  Google Scholar 

  10. Shah SR, Gupta AD, Sharma A, et al. (2003) Acute superior mesenteric vein thrombosis associated with factor V Leiden gene mutation. J Assoc Physicians India 51:611–613

    PubMed  CAS  Google Scholar 

  11. Tomizuka H, Hatake K, Kitagawa S, et al. (1999) Portal vein thrombosis in paroxysmal nocturnal haemoglobinuria. Acta Haematol 101:149–152

    Article  PubMed  CAS  Google Scholar 

  12. Gayer G, Zandman-Goddard G, Raanani P, et al. (2001) Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT. Abdom Imaging 26:414–419

    Article  PubMed  CAS  Google Scholar 

  13. McBane RD, Panneton JM, Bate WW (2001) Aortic thrombosis as a complication of paroxysmal nocturnal hemoglobinuria. Circulation 104:e1–e2

    Article  PubMed  Google Scholar 

  14. Hilimen PH, Lewis SM, Bessler M, et al. (1995) Natural history of paroxysmal nocturnal hemoglobinuria. New Engl J Med 333:1253–1258

    Article  Google Scholar 

  15. Hartman RC, Luther AB, Jenkins DE, et al. (1980) Fulminent hepatic venous thrombosis (Budd–Chiari syndrome) in paroxysmal nocturnal hemoglobinuria: definition of a medical emergency. Johns Hopkins Med J 146:247–254

    Google Scholar 

  16. Witz M, Kantarovsky A, Morag B, et al. (1996) Renal vein occlusion: a review. J Urol 155:1173–1179

    Article  PubMed  CAS  Google Scholar 

  17. Socie G, Mary J, Gramont A, et al. (1996) Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. Lancet 348:573–577

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shindo, S., Motohashi, S., Kaga, S. et al. Paroxysmal nocturnal hemoglobinuria: complete resolution of an occluding inferior vena caval thrombus. Abdom Imaging 32, 754–757 (2007). https://doi.org/10.1007/s00261-007-9184-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-007-9184-6

Keywords

Navigation