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Risk Factors for Premature Mortality


A comprehensive clinical assessment is crucial to determine the risk for morbidities and premature mortality in your patient with PNH2,3,5,6,7

Prevalence of TE in different PNH granulocyte clone size categories8

  • Monitoring of patients with small PNH clones is essential since clone size increased in 40% (10/25) of patients with PNH clone size between 0.11% and 10%8

Hemolysis is a risk factor for TE and premature mortality10

Prevalence of TE in different PNH granulocyte clone size categories8

  • Patients with PNH with hemolysis (LDH ≥1.5x ULN) at diagnosis had a mortality rate nearly 5-fold greater than the age- and gender-matched general population (P<0.001)9
  • TE is strongly associated with increased risk for premature mortality in patients with PNH (P<0.001)4

Hemolysis (LDH ≥1.5x ULN) is a statistically significant indicator of TE risk (P<0.013)4

  • 96% of patients with TE were detected using LDH ≥1.5x ULN as a threshold
  • Parameters such as age, BMF history, clone size, WBC count, platelet count, and hemoglobin level were not significant indicators of TE risk (P-value range: 0.111-0.981)

Elevated hemolysis alone, and in association with clinical symptoms, increases TE risk4

Prevalence of TE in different PNH granulocyte clone size categories8

  • Incidences of abdominal pain, chest pain, and dyspnea were significantly increased in patients with PNH and TE compared with those without TE4
  • Elevated hemolysis, with any one of those clinical symptoms, was associated with a greater increased risk for TE than elevated hemolysis or clinical symptoms alone4

Patients are at risk for TE regardless of clone size4

Prevalence of TE in different PNH granulocyte clone size categories8

  • Even patients with smaller clone sizes can experience thrombosis, and there was no evidence of any association between clone size category and risk of experiencing TE (P=0.843)4

In PNH, fatigue and impaired quality of life (QoL) are independent of clone size11

Prevalence of TE in different PNH granulocyte clone size categories8

  • Even patients with clone size <10% experienced substantial impact on QoL11

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References: 1. Rother RP, Bell L, Hillmen P, et al. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005;293:1653-1662. 2. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2007;137:181-192. 3. Nishimura J-I, Kanakura Y, Ware RE, et al. Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine. 2004;83:193-207. 4. Lee JW, Jang JH, Kim JS, et al. Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. Int J Hematol. 2013;97:749-757. 5. Parker C, Omine M, Richards S, et al; for International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709. 6. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 7. Weitz I, Meyers G, Lamy T, et al. Cross-sectional validation study of patient-reported outcomes in patients with paroxysmal nocturnal haemoglobinuria. Intern Med J. 2013;43:298-307. 8. Movalia M, lllingworth A, Weitz I, et al. Poster presented at the 53rd Annual Meeting of the American Society of Hematology; December 10-13, 2011; San Diego, CA. Abstract 1033. 9. Lee JW, Jang JH, Kim JS, et al. Uncontrolled complement activation and the resulting chronic hemolysis as measured by LDH serum level at diagnosis as predictor of thrombotic complications and mortality in a large cohort of patients with paroxysmal nocturnal hemoglobinuria (PNH). Blood. 2011;118: Abstract 3166. 10. Kim JS, Lee JW, Yoon SS, et al. Association between elevated hemolysis at diagnosis and early mortality and risk of thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) patients with cytopenia. Blood. 2010;116: Abstract 4241. 11. Urbano-Ispizua A, Schrezenmeier H, Brodsky R, et al. Evaluation of paroxysmal nocturnal hemoglobinuria disease burden in patients enrolled in the International PNH Registry. In: Abstracts of the 15th Congress of the European Hematology Association; June 10-13, 2010; Barcelona, Spain. Abstract 1022.