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It depends on the variability of her haemolysis. If this is a steady state condition, HbA1c could be used for on-going monitoring - BUT it would be necessary to do some extra testing in the early phases to establish what her personal reference range should be.
 





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From: Clinical biochemistry discussion list [mailto:[log in to unmask]] On Behalf Of [log in to unmask]
Sent: 07 August 2013 1:27 AM
To: [log in to unmask]
Subject: HbA1c and pyruvate kinase deficiency


Hello all,
I would greatly appreciate this group's opinion on the possibility to use HbA1c in a 14 year old girl with type 1 diabetes and pyruvate kinase deficiency.
Usual guidelines say that in all hemolytic conditions HbA1c could be falsely underestimated due to to short RBC lifespan.
However there are various articles (see below) that say that in PK deficiency it is mostly reticulocytes that are sequestered in the spleen and that older RBC's have a near normal lifespan:

Selective reticulocyte destruction in erythrocyte pyruvate kinase deficiency
William C. Mentzer Jr., Robert L. Baehner, Holger Schmidt-Schönbein, Stephen H. Robinson, David G. Nathan
Published in Volume 50, Issue 3
J Clin Invest. 1971; 50(3):688-699 doi:10.1172/JCI106539

Red cell pyruvate kinase deficiency: molecular and clinical aspects
Alberto Zanella,1 Elisa Fermo,1 Paola Bianchi,1 and Giovanna Valentini2,3
2005 Blackwell Publishing Ltd, British Journal of Haematology, 130, 11-25

Could this mean that HbA1c can be used, with caution, in this condition? Any experiences?

Antonio Croce
Sondrio Hospital Laboratory, Italy
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