CHRONIC MYELOMONOCYTIC LEUKEMIA ASSOCIATED WITH HEREDITARY PYRUVATE-KINASE DEFICIENCY AND MULTIPLE ACQUIRED ERYTHROCYTE ABNORMALITIES

被引:6
作者
VIVESCORRONS, JL [1 ]
FLORENSA, L [1 ]
MUNCUNILL, J [1 ]
NOMDEDEU, B [1 ]
ROZMAN, C [1 ]
机构
[1] UNIV BARCELONA,HOSP CLIN & PROVINCIAL,CENT HEMATOL LAB,BARCELONA,SPAIN
关键词
Acquired erythroenzymopathies; Chronic myelomonocytic leukemia; Pyruvate kinase deficiency;
D O I
10.1159/000207651
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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. © 1979 S. Karger AG, Basel.
引用
收藏
页码:168 / 174
页数:7
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