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

More documents

Recommendations

Info

Paroxysmal Nocturnal Haemoglobinuria • Firstly, a few facts: • It’s s not paroxysmal – Even in the absence of symptoms, destructive progression of haemolysis is ongoing • It’s s not nocturnal – Haemolysis in PNH is subtle and constant, 24 hours a day • Haemoglobinuria is a less commonly seen complication – ¾ patients present without haemoglobinuria 1 1. International PNH Interest Group. Blood. 2005;106:3699-3709. August_12_2010Gbl 4
The Defect in PNH The Somatic Mutation of the PIG A gene prevents all GPI anchored proteins from binding to cell surface CD59 • Forms a defensive shield for RBCs from complement-mediated mediated lysis • Inhibits the assembly of the membrane attack complex CD55 • Prevents formation and augments instability of the C3 convertases, attenuating the complement cascade CD59 GPI-anchor CD55 1. Adapted from: Johnson RJ et al. J Clin Pathol: Mol Pathol. 2002;55:145-152. 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. August_12_2010Gbl 5
Page 1 and 2: Paroxysmal Nocturnal Haemoglobinuri
Page 3: Paroxysmal Nocturnal Haemoglobinuri
Page 7 and 8: PNH is a Progressive Disease of Chr
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

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

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?