Paroxysmal nocturnal haemoglobinuria and its complications

File:1GZX Haemoglobin.png

Look how this article begins:

“A 24-year-old medical editor presented to her primary care physician with fevers, epigastric pain, nausea, and vomiting upon returning from a business trip…”

I’d be surprised if your first thought was PNH. In fact, not only is PNH quite rare but the ‘classic triad’ of symptoms is not always present – intravascular haemolysis, thrombosis, and cytopaenia. The key therapy is eculizumab, which really revolutionized therapy, thanks to the SHEPHERD and TRIUMPH trials. This article in Blood nicely takes us through an interesting case study and a great discussion on PNH and its complications. Definitely worth a read – check it out here.

 

source | Blood

image | Richard Wheeler

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