How is thromboembolism treated in paroxysmal nocturnal hemoglobinuria (PNH)?

Updated: May 20, 2021
  • Author: Emmanuel C Besa, MD; Chief Editor: Sara J Grethlein, MD, FACP  more...
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Answer

Patients with PNH who develop acute thrombosis should immediately be started on eculizumab or ravulizumab, if they are not already taking it, as this reduces the risk of thrombosis extension or recurrence. [13] Otherwise, management of thrombotic complications follows standard principles, including using heparin emergently, then maintenance therapy with an oral anticoagulant, such as warfarin. Sometimes, heparin can exacerbate the thrombotic problem, possibly by activating complement. This can be prevented by using inhibitors of the cyclooxygenase system such as aspirin, ibuprofen, or sulfinpyrazone.

Primary prophylaxis of thromboembolism for patients with PNH has been advocated. Whether this approach is safe and effective in all patients with PNH remains controversial, however.


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