What is the median survival in patients with 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

The median survival after diagnosis was 10 years in a series of 80 consecutive patients seen at the Hammersmith Hospital in London who were treated with supportive measures, such as oral anticoagulant therapy after an established thrombosis, and transfusions. [17] Sixty patients died; of 48 patients whose cause of death was known, 28 died from venous thrombosis or hemorrhage. Thirty-one individuals (39%) had one or more episodes of venous thrombosis during their illness. [17] No leukemic transformations occurred in this series.

Twenty-two of the 80 patients (28%) survived for 25 years. [17] Of the 35 patients who survived for 10 years or more, 12 had spontaneous clinical recovery; no PNH-affected cells were found among the RBCs or neutrophils during their prolonged remission, but a few PNH-affected lymphocytes were detectable in 3 of 4 patients tested. [17]


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