Paroxysmal Nocturnal Haemoglobinuria (PNH) Dr Conal McConville ...
Paroxysmal Nocturnal Haemoglobinuria (PNH) Dr Conal McConville ...
Paroxysmal Nocturnal Haemoglobinuria (PNH) Dr Conal McConville ...
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<strong>Paroxysmal</strong> <strong>Nocturnal</strong> <strong>Haemoglobinuria</strong>:<br />
A Chronic Disabling and Life-Threatening Disease<br />
• Estimated 4,000 –<br />
6,000 patients in U.S. 1<br />
• 5 year mortality: 35% 2<br />
• Diagnosed at all<br />
Ages – Median age<br />
early 30’s 3,4<br />
• Quality of life<br />
diminished 5<br />
• Progressive disease 2-4<br />
Patients Surviving (%)<br />
100<br />
80<br />
60<br />
40<br />
20<br />
0<br />
Actuarial Survival From the Time of<br />
Diagnosis in 80 Patients With <strong>PNH</strong> 2<br />
0 5 10 15 20 25<br />
Years After Diagnosis<br />
Age- and sex-<br />
matched controls<br />
The expected survival of an age- and sex-matched control group is shown for comparison<br />
(Hillmen et al 1995). In a patient population where ½ the patients have
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