Prevalence and management of iron overload in pyruvate kinase deficiency: report from the Pyruvate Kinase Deficiency Natural History Study
UMass Chan Affiliations
Department of Pediatrics, Division of Hematology OncologyDocument Type
Accepted ManuscriptPublication Date
2018-09-13Keywords
ChelationIron Overload
Pyruvate Kinase Deficiency
Red Cell Enzyme Abnormalities
Splenectomy
Enzymes and Coenzymes
Hematology
Hemic and Lymphatic Diseases
Inorganic Chemicals
Nutritional and Metabolic Diseases
Therapeutics
Metadata
Show full item recordAbstract
Pyruvate kinase (PK) deficiency is the most common red cell glycolytic enzyme defect causing hereditary non-spherocytic hemolytic anemia. Current treatments are mainly supportive and include red cell transfusions and splenectomy.11 Regular red cell transfusions are known to result in iron overload; however, the prevalence and spectrum of transfusion-independent iron overload in the overall PK deficient population has not been well defined. This analysis describes the prevalence and clinical characteristics of iron overload in patients enrolled in the PK Deficiency Natural History Study (NHS) with a focus on those patients who are not regularly transfused.2Source
Haematologica. 2018 Sep 13. pii: haematol.2018.196295. doi: 10.3324/haematol.2018.196295. [Epub ahead of print] Link to article on publisher's site
DOI
10.3324/haematol.2018.196295Permanent Link to this Item
http://hdl.handle.net/20.500.14038/40788PubMed ID
30213831Notes
Full author list omitted for brevity. For the full list of authors, see article.
Related Resources
Rights
Copyright © 2018, Ferrata Storti Foundation. Authors' accepted manuscript posted as allowed by the publisher's author rights policy at http://www.haematologica.org/information-for-authors.ae974a485f413a2113503eed53cd6c53
10.3324/haematol.2018.196295