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

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Thrombosis Chronic Kidney Disease Fatigue / Impaired QOL Haemolysis Causes Fatigue Independent of Anemia Pulmonary Hypertension • Rosse (Hoffman-Haematology) 1 – “Many patients note a feeling of fatigue that may be disabling during periods of haemoglobinuria.” – This is not related to haemoglobin level (anaemia), as it disappears when the haemoglobinuria stops.” • Brodsky (Hoffman-Haematology) 2 – “PNH patients frequently complain of disabling fatigue that is often out of proportion to the degree of anaemia.” • Multivariate analysis indicates haemolysis drives fatigue in PNH – not anaemia 3 1. Rosse W. Paroxysmal nocturnal haemoglobinuria In: R Hoffman; EJ Benz; SJ Shattil et al., eds. Hematology: Basic Principles and Practice. 3rd ed. New York: Churchill-Livingstone; 2000:331-342. 2. Brodsky RA. Paroxysmal nocturnal haemoglobinuria. In: R Hoffman; EJ Benz; SJ Shattil et al., eds. Hematology. Basic Principles and Practice. 4th ed. Philadelphia: Elsevier Churchill Livingstone; 2005:419-427. 3. Hill A et al. Haematologica. 2008;93(s1):359. Abstract 0903 and Presentation at: European Hematology Association 15 th Annual Meeting; June 15 th , 2008; Copenhagen, Denmark. August_12_2010Gbl 18
Thrombosis Chronic Kidney Disease Fatigue / Impaired QOL Pulmonary Hypertension Pain is a Common Symptom in PNH Patients Pain, No treatment Medical Intervention Pain, Treatment Opioids Other No pain NSAID (n=286) Almost 3 out of 5 (58%) patients reported significant pain 47% of patients with pain required medical intervention South Korean National Registry Lee JW et al. Hematologica 2010. 95 (s2): Abstract #506. August_12_2010Gbl 19
Page 1 and 2: Paroxysmal Nocturnal Haemoglobinuri
Page 3 and 4: Paroxysmal Nocturnal Haemoglobinuri
Page 5 and 6: The Defect in PNH The Somatic Mutat
Page 7 and 8: PNH is a Progressive Disease of Chr
Page 9 and 10: Thrombosis Chronic Kidney Disease F
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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

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

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