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

Autor:	Daniel Painter, Louise Arnold, Claire E McKinley, Daniel Payne, Darren J. Newton, Morag Griffin, Rachael Jones, Anita J. Dickinson, Anita Hill, Stephen J. Richards, Eve Roman, Richard Kelly, Alexandra Pike, Alexandra Smith, Talha Munir, Peter Hillmen, Petra Muus
Rok vydání:	2021
Předmět:	Adult Male medicine.medical_specialty Adolescent Population Prevalence Hemoglobinuria Paroxysmal Population based History 21st Century Immunophenotyping Young Adult hemic and lymphatic diseases Internal medicine medicine Retrospective analysis Humans education Child Aged Retrospective Studies Aged 80 and over education.field_of_study business.industry Incidence (epidemiology) Incidence Anemia Aplastic Hematology General Medicine Syndrome Middle Aged United Kingdom Child Preschool Population Surveillance Female Paroxysmal nocturnal haemoglobinuria business Haematological malignancy Biomarkers Rare disease
Zdroj:	European journal of haematologyREFERENCES. 107(2)
ISSN:	1600-0609
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4170186c216e39250ab9b3fcc00696d6 https://pubmed.ncbi.nlm.nih.gov/34060690 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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