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Paroxysmal Nocturnal Haemoglobinuria (PNH) Dr Conal McConville ...

Paroxysmal Nocturnal Haemoglobinuria (PNH) Dr Conal McConville ...

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Thrombosis<br />

Chronic Kidney Disease<br />

Fatigue / Impaired QOL<br />

Haemolysis Causes Fatigue<br />

Independent of Anemia<br />

Pulmonary Hypertension<br />

• Rosse (Hoffman-Haematology) 1<br />

– “Many patients note a feeling of fatigue that may be<br />

disabling during periods of haemoglobinuria.”<br />

– This is not related to haemoglobin level (anaemia), as it<br />

disappears when the haemoglobinuria stops.”<br />

• Brodsky (Hoffman-Haematology) 2<br />

– “<strong>PNH</strong> patients frequently complain of disabling fatigue<br />

that is often out of proportion to the degree of anaemia.”<br />

• Multivariate analysis indicates haemolysis drives fatigue in<br />

<strong>PNH</strong> – not anaemia 3<br />

1. Rosse W. <strong>Paroxysmal</strong> nocturnal haemoglobinuria In: R Hoffman; EJ Benz; SJ Shattil et al., eds. Hematology: Basic Principles and Practice. 3rd ed. New<br />

York: Churchill-Livingstone; 2000:331-342. 2. Brodsky RA. <strong>Paroxysmal</strong> nocturnal haemoglobinuria. In: R Hoffman; EJ Benz; SJ Shattil et al., eds. Hematology.<br />

Basic Principles and Practice. 4th ed. Philadelphia: Elsevier Churchill Livingstone; 2005:419-427. 3. Hill A et al. Haematologica. 2008;93(s1):359. Abstract<br />

0903 and Presentation at: European Hematology Association 15 th Annual Meeting; June 15 th , 2008; Copenhagen, Denmark.<br />

August_12_2010Gbl<br />

18

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