This site is intended for US audiences only. If you are a resident of the European Union, please click here for the EU version of our site.
Thrombosis
Thrombosis: The Leading Cause of Mortality in PNH1,2
Venous and arterial thromboses account for approximately 40% to 67% of PNH-related deaths.3
- First thrombotic event increases risk of death 5- to 10-fold3
- First thrombotic event can be fatal3,4
- Pulmonary embolism (PE) or deep vein thrombosis (DVT) is the most common clinical presentation1,3
Risk of thrombosis exists regardless of clone size, lack of transfusion history, severity of anemia, anticoagulant therapy, or level of hemolysis.3,5,6
- 28% of never-transfused patients with PNH had evidence of thromboembolism (TE)7
- Median time to thrombotic event was ~2 years from diagnosis8
Symptoms Frequently Experienced by PNH Patients Are Predictive of TE and Mortality9
Study description: A retrospective analysis of the medical charts of 286 patients with PNH in
a national registry.
Both Venous and Arterial Thrombosis Events Occur With High Frequency in PNH
- Occurrence of TE was independent of granulocyte clone size9
- In a national registry, 39% of TE events occurred in arterial sites9
- PNH TE occurs in typical and atypical sites9
- DVT, mesenteric, and PE most common
- Budd-Chiari, renal, and dermal less common
Precursors of Thromboembolism May Help Identify Those at Risk
- Early research shows promise in using markers of thrombin generation and inflammation, which may be associated with increased risk of thromboembolism. These markers include10:
- D-dimers
- 82% of all patients with PNH had elevated D-dimers
- IL-6
- Thrombin-antithrombin (TAT) complex
- D-dimers
Next: Chronic Kidney Disease
1. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. N Engl J Med. 1995;333:1253-1258.
2. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Lancet. 1996;348:573-577. 3. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 4. Audebert HJ, Planck J, Eisenburg M, Schrezenmeier H, Haberl RL. J Neurol. 2005;252:1379-1386. 5. Hill A, Richards SJ, Hillmen P. Br J Haematol. 2007;137:181-192. 6. Bessler M, Schrezenmeier H, Maciejewski JP, et al. [ASH abstract]. Blood. 2007;110: Abstract 840. 7. Muus P, Risitano AM, Castro-Malaspina HR, Jones CM, Fuller S, Socié G. [ASH abstract]. Blood. 2009;114: Abstract 4029.
8. Peffault de Latour R, Mary JY, Salanoubat C, et al. Blood. 2008;112:3099-3106. 9. Lee, JW, Jang JH, Lee JH, Yoon SS, et al. In: Posters of the 15th Congress of the European Hematology Association; June 10-13, 2010; Barcelona, Spain. Abstract 0505. 10. Weitz IC. Thrombosis Res. 2010;125(suppl 2):S106-S107.
2. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Lancet. 1996;348:573-577. 3. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 4. Audebert HJ, Planck J, Eisenburg M, Schrezenmeier H, Haberl RL. J Neurol. 2005;252:1379-1386. 5. Hill A, Richards SJ, Hillmen P. Br J Haematol. 2007;137:181-192. 6. Bessler M, Schrezenmeier H, Maciejewski JP, et al. [ASH abstract]. Blood. 2007;110: Abstract 840. 7. Muus P, Risitano AM, Castro-Malaspina HR, Jones CM, Fuller S, Socié G. [ASH abstract]. Blood. 2009;114: Abstract 4029.
8. Peffault de Latour R, Mary JY, Salanoubat C, et al. Blood. 2008;112:3099-3106. 9. Lee, JW, Jang JH, Lee JH, Yoon SS, et al. In: Posters of the 15th Congress of the European Hematology Association; June 10-13, 2010; Barcelona, Spain. Abstract 0505. 10. Weitz IC. Thrombosis Res. 2010;125(suppl 2):S106-S107.
Dr Wendell Rosse explains how the pathophysiology of PNH can lead to thrombosis.
Visit the PNH Education Center for case studies, videos, and brochures dealing with a wide range of PNH topics.