Paroxysmal nocturnal hemoglobinuria natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

If left untreated, patients with paroxysmal nocturnal hemoglobinuria may progress to develop thrombosis which is a main cause of death in PNH. Common complications include intracranial thrombosis, splenic vein thrombosis, and portal vein thrombosis. Prognosis of paroxysmal nocturnal hemoglobinuria is good as long as anti-complemant therapy eculizumab is taken regularly.

Natural History, Complications, and Prognosis

Natural History

• If left untreated, patients with paroxysmal nocturnal hemoglobinuria may progress to develop thrombosis which is a main cause of death in PNH.
• PNH is associated with high morbidity and mortality rate if appropriate treatment is not administrated regularly.

Complications

• Common complications of paroxysmal nocturnal hemoglobinuria include:
  • Intracranial thrombosis
  • Splenic vein thrombosis
  • Portal vein thrombosis

Prognosis

• Prognosis of paroxysmal nocturnal hemoglobinuria is good as long as anti-complemant therapy eculizumab is taken regularly.
• Eculizumab has shown decrease in the risk of thrombosis which is a main cause of death in patients with PNH.