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

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Historically Viewed as a Hemolytic Anemia Normal red blood cells are protected from complement attack by a shield of terminal complement inhibitors Without this protective complement inhibitor shield, PNH red blood cells are destroyed Complement Activation Intact RBC Reduced Free Haemoglobin Red Cell Mass Anaemia 1. International PNH Interest Group. Blood. 2005;106:3699-3709. 2. Brodsky R Paroxysmal Nocturnal Haemoglobinuria. In: Hematology - Basic Principles 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. 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. August_12_2010Gbl 6
PNH is a Progressive Disease of Chronic Haemolysis Normal red blood cells Without this protective Thrombosis are protected from complement inhibitor complement attack by shield, PNH red blood a shield of terminal cells are destroyed Renal Failure complement inhibitors Pulmonary Hypertension Significant Impact on Survival Complement Activation Abdominal Pain Dyspnoea Intact RBC Free Haemoglobin Dysphagia Fatigue Significant Impact on Morbidity Haemoglobinuria Anaemia Erectile Dysfunction 1. International PNH Interest Group. Blood. 2005;106:3699-3709. 2. Brodsky R Paroxysmal Nocturnal Haemoglobinuria. In: Hematology - Basic Principles 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. 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 (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. August_12_2010Gbl 7
Page 1 and 2: Paroxysmal Nocturnal Haemoglobinuri
Page 3 and 4: Paroxysmal Nocturnal Haemoglobinuri
Page 5: The Defect in PNH The Somatic Mutat
Page 9 and 10: Thrombosis Chronic Kidney Disease F
Page 23 and 24: Common Symptoms of Haemolysis Signa
Page 25 and 26: Historical Management of PNH Bone M
Page 27 and 28: SOLIRIS ® Blocks Terminal Compleme
Page 29 and 30: Adverse Reactions Reported in ≥ 5
Page 31: August 12_10_2010Gbl Thank you Than

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