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Chronic Myelomonocytic Leukemia Associated with Hereditary Pyruvate Kinase Deficiency and Multiple Acquired Erythrocyte Abnormalities

Chronic Myelomonocytic Leukemia Associated with Hereditary Pyruvate Kinase Deficiency and... 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-phospho-gluconate 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Chronic Myelomonocytic Leukemia Associated with Hereditary Pyruvate Kinase Deficiency and Multiple Acquired Erythrocyte Abnormalities

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Publisher
Karger
Copyright
© 1979 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000207651
Publisher site
See Article on Publisher Site

Abstract

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-phospho-gluconate 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.

Journal

Acta HaematologicaKarger

Published: Jan 1, 1979

Keywords: Acquired erythroenzymopathies; Chronic myelomonocytic leukemia; Pyruvate kinase deficiency

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