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The incidence and prevalence of patients with paroxysmal nocturnal haemoglobinuria and aplastic anaemia PNH syndrome: a retrospective analysis of the UK’s population-based haematological malignancy research network 2004-2018

Research output: Contribution to journalArticlepeer-review

Published copy (DOI)

Author(s)

  • Stephen J. Richards
  • Daniel Painter
  • Anita J. Dickinson
  • Morag Griffin
  • Talha Munir
  • Louise Arnold
  • Daniel Payne
  • Alexandra Pike
  • Petra Muus
  • Anita Hill
  • Darren J. Newton
  • Claire McKinley
  • Rachael Jones
  • Richard Kelly
  • Alexandra Gwen Smith
  • Eve Roman
  • Peter Hillmen

Department/unit(s)

Publication details

JournalEuropean Journal of Haematology
DateAccepted/In press - 27 Apr 2021
DateE-pub ahead of print - 9 Jun 2021
DatePublished (current) - 7 Jul 2021
Issue number2
Volume107
Number of pages8
Pages (from-to)211-218
Early online date9/06/21
Original languageEnglish

Abstract

Abstract Objectives A retrospective population-based study to determine the incidence and prevalence of patients with the rare blood disease paroxysmal nocturnal haemoglobinuria (PNH). Methods All patients were identified by flow cytometric detection of blood cells deficient in glycosylphosphatidylinositol (GPI) linked proteins at a single diagnostic reference laboratory that serves the Yorkshire based, Haematological Malignancy Research Network (HMRN) with a population of 3.8 million. Results One hundred and ninety-seven patients with detectable PNH clones at a level of >0.01% in at least two lineages of cells (neutrophils, monocytes and/or red cells) were identified over a 15-year period (2004-2018). Of these, 88% had aplastic anaemia (AA), 8% classical PNH and 3% myelodysplastic syndrome. The overall incidence rate was estimated at 0.35 cases per 100 000 people per year. This equates to 220 cases newly diagnosed in the United Kingdom each year. The overall prevalence rate was 3.81 per 100 000, this equates to an estimated 2400 prevalent cases in the UK. The overall and relative 5-year survival rates were 72% and 82.7%, respectively. Conclusions This study showed that classical haemolytic PNH is a rare disease and represents only a small proportion overall of patients with detectable PNH cells, the majority of which have aplastic anaemia.

Bibliographical note

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    Research areas

  • aplastic anaemia, incidence, paroxysmal nocturnal haemoglobinuria, prevalence

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