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IPIG 2025 | The clinical problem of thrombosis in patients with PNH
Jeff Szer, MBBS, FRACP, Peter MacCallum Cancer Centre, Melbourne, Australia, comments on the clinical challenge of thrombosis in patients with paroxysmal nocturnal hemoglobinuria (PNH), stating that thrombosis often occurs in unusual locations and can be fatal if not recognized and managed early. Dr Szer emphasizes the importance of clinicians identifying symptoms and signs of PNH, as thrombosis-related death may occur due to underlying undiagnosed PNH. This interview took place at the 2nd International PNH Interest Group (IPIG) Conference in Paris, France.
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Transcript
Yes, so we have two… three sort of levels of thrombotic risk in PNH. It’s been described by many as the most vicious thrombophilic disorder. So there are very few conditions associated with thrombosis that have a higher risk of thrombosis than PNH. And it’s not the majority of patients, but it’s a very significant minority, and is often the presenting feature, and often in unusual locations...
Yes, so we have two… three sort of levels of thrombotic risk in PNH. It’s been described by many as the most vicious thrombophilic disorder. So there are very few conditions associated with thrombosis that have a higher risk of thrombosis than PNH. And it’s not the majority of patients, but it’s a very significant minority, and is often the presenting feature, and often in unusual locations. So the risk is high, and the major mitigating factor is the introduction of anti-complement therapy for the patients who actually survived that first instance. We don’t know how many thromboses have occurred in patients that were fatal in a patient with underlying PNH, so that’s why the educative aspect of meetings such as the one we’re at now and many others around this ultra-rare disease are so important so that symptoms and signs and laboratory features of PNH can be recognised before a potentially fatal complication. We do know that thrombosis is the major cause of death in PNH patients and results in the poor prognosis of its untreated state.
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Disclosures
Financial relationship (not shares) with Alexion, Sobi, Novartis.
