Document Detail


Erythrocyte populations in pyruvate kinase deficiency anaemia following splenectomy. II. Cell deformability.
MedLine Citation:
PMID:  666978     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The deformability of three previously described morphological populations of erythrocytes from splenectomized pyruvate kinase (PK) deficient patients was assessed using both Nucleopore filtration and micropipette techniques. Mean deformability was decreased in fresh cells from all patients studied. Selective measurements indicated that immature lobulated reticulocytes in each patient were the least deformable cells, followed by bizarre echinocytes characterized by a hyperviscous behaviour, and by thin macrocytic discocytes whose values were closest to those of control erythrocytes. We conclude that poorly deformable reticulocytes in these patients, after initial trapping in the spleen, are unable to return to the circulation because of their inordinate dependence on mitochondrial oxydative phosphorylation for maintenance of a sufficient intracellular concentration of adenosine triphosphate (ATP); hence their rapid and selective destruction. Splenectomy therefore may be beneficial by allowing such cells to mature more slowly in the circulation of these patients until they develop crenation and hyperviscosity, two manifestations of profound ATP depletion possibly leading to destruction by the liver.
Authors:
P F Leblond; L Coulombe; J Lyonnais
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  British journal of haematology     Volume:  39     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  1978 May 
Date Detail:
Created Date:  1978-09-25     Completed Date:  1978-09-25     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  63-70     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Hemolytic / blood*,  physiopathology,  therapy
Child
Erythrocytes / physiology*
Female
Humans
Male
Pyruvate Kinase / deficiency*
Reticulocytes / physiology
Rheology
Splenectomy*
Chemical
Reg. No./Substance:
EC 2.7.1.40/Pyruvate Kinase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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