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

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

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<strong>PNH</strong> is a Progressive Disease of<br />

Chronic Haemolysis<br />

Normal red blood cells<br />

Without this protective<br />

Thrombosis<br />

are protected from<br />

complement inhibitor<br />

complement attack by<br />

shield, <strong>PNH</strong> red blood<br />

a shield of terminal<br />

cells are destroyed<br />

Renal Failure<br />

complement inhibitors<br />

Pulmonary Hypertension<br />

Significant<br />

Impact on<br />

Survival<br />

Complement<br />

Activation<br />

Abdominal Pain<br />

Dyspnoea<br />

Intact RBC<br />

Free Haemoglobin<br />

Dysphagia<br />

Fatigue<br />

Significant<br />

Impact on<br />

Morbidity<br />

<strong>Haemoglobinuria</strong><br />

Anaemia<br />

Erectile Dysfunction<br />

1. International <strong>PNH</strong> Interest Group. Blood. 2005;106:3699-3709. 2. Brodsky R <strong>Paroxysmal</strong> <strong>Nocturnal</strong> <strong>Haemoglobinuria</strong>. In: Hematology - Basic Principles<br />

and Practices. 4th ed. R Hoffman; EJ Benz; S Shattil et al, eds. Philadelphia, PA: Elsevier Churchill Livingstone; 2005; p. 419-427. 3. Rother RP et al. JAMA.<br />

2005;293:1653-1662. 4. Socie G et al. Lancet. 1996;348:573-577. 5. Hill A et al. Br J Haematol. 2007;137:181-92. 6. Lee JW et al. Hematologica 2010. 95<br />

(s2): Abstract #505 and 506. 7. Hill A et al. Br J Haematol. 2010; May;149(3):414-25. 8. Hillmen P et al. Am. J. Hematol. 2010; 85:553–559.<br />

August_12_2010Gbl<br />

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