PAROXYSMAL NOCTURNAL HAEMOGLOBINURIA (PNH)
is a rare, life-threatening condition driven by complement-mediated intravascular haemolysis.1-3
Reaching a diagnosis of PNH may be delayed due its rarity and the overlap of symptoms with other conditions.1
If you suspect PNH, the diagnostic pathway on this website may be a useful guide to follow.
Adapted from Roth A et al. 2018.1 aParker 2005; bPeffault de Latour 2008, Brodsky 2009, Valent 2012; cNICE 2015. †
An unexplained persistent cytopaenia in patients in whom (minimal) diagnostic criteria for MDS are not fulfilled. *DVT and/or PE in a patient with no antecedent major clinical risk factor for VTE that is not provoked by surgery,
trauma, immobilisation, hormonal therapy (oral contraceptive or hormone replacement therapy) or active cancer. **Unusual sites include
hepatic veins (Budd Chiari syndrome), other splanchnic veins (portal, splenic) cerebral vein and dermal veins.1
DVT: deep vein thrombosis; FLAER: fluorescein-labelled proaerolysin; LDH: lactate dehydrogenase; MDS: myelodysplastic syndrome; PE: pulmonary embolism; VTE: venous thromboembolism.
THE PNH MANAGEMENT CYCLE
THE PATH TO DIAGNOSING PNH MAY BE COMPLICATED,
BUT THE TREATMENT DOESN’T NEED TO BE.
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References: 1. Roth A et al. Eur J Haematol 2018;1-9. 2. Borowitz MJ et al. Cytometry B Clin Cytom 2010;78:211–230. 3. Parker C et al. Blood 2005;106:3699–3709.