Chronic myelomonocytic leukemia associated with hereditary pyruvate kinase deficiency and multiple acquired erythrocyte abnormalities

Acta Haematologica
J L Vives-CorronsC Rozman


A congenital erythrocyte pyruvate kinase (PK) deficiency was found in a 72-year old female patient with chronic myelomonocytic leukemia (CMML). Erythrocyte PK deficiency was associated with an increase in the activity of hexokinase, 6-phosphogluconate dehydrogenase and glutathione peroxidase in erythrocytes as well as a decrease in acetylcholinesterase, glutathione reductase and glucosephosphate isomerase activities. The enzymatic abnormalities were accompanied by alterations in hemoglobin and in i antigen content of erythrocyte membrane. In addition, bone marrow ultrastructural studies showed dyshemopoietic changes in all blood cell lines and especially in erythroblasts. The present findings confirm the close relationship between CMML and acquired dyserythropoietic syndromes and constitute a new observation of the infrequent association of hereditary erythrocyte enzymopathies and leukemia. A survey of the literature is presented.


Sep 5, 2001·Leukemia Research·L G Boros, R D Williams
Jan 1, 1981·Blut·S WoessnerJ Sans-Sabrafen
Jun 20, 2015·American Journal of Hematology·Rachael F GraceBertil Glader

Related Concepts

Autocrine Motility Factor
Selenoglutathione Peroxidase
Glutathione Reductase
Hexokinase 2
Leukemia, Monocytic, Chronic
Phosphogluconate Dehydrogenase
R-Type Pyruvate Kinase

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