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Erythrocyte Populations in Pyruvate Kinase Deficiency Anaemia following Splenectomy
Author(s) -
Leblond Pierre F.,
Lyonnais Jean,
Delage JeanMarie
Publication year - 1978
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1978.tb07127.x
Subject(s) - echinocyte , pyruvate kinase deficiency , pyruvate kinase , red cell , splenectomy , red blood cell , intracellular , biology , immunology , pathology , medicine , biochemistry , spleen , metabolism , glycolysis
S ummary . Light and scanning electron microscopic examination of fresh erythrocytes from the blood of four patients splenectomized for severe pyruvate kinase (PK) deficiency haemolytic anaemia has revealed three morphologically different cell populations. Most striking in these patients was the presence of crenated red cells (30–50% of total) which fit current criteria for definition as cchinocytes, but also with unusual surface retraction compatible with the existence of intracellular shrinkage, a phenomenon known to occur after incubation of PK deficient red cells in vitro. Additional cell types include immature reticulocytes (7–8% of total) and thin macrocytic discocytes (45–65% of total) none of which are considered specific for the disease. These morphological findings are in sharp contrast with the paucity of red cell shape abnormalities usually described in PK deficient patients before splenectomy and may provide insight into the mechanisms leading to accelerated erythrocyte destruction in this disease.