Changing erythrocyte populations in juvenile chronic myelocytic leukemia: evidence for disordered regulation

George J Dover, S. H. Boyer, W. H. Zinkham, Haig Kazazian, D. J. Pinney, A. Sigler

Research output: Contribution to journalArticle

Abstract

Fetal and adult erythrocyte characteristics were studied serially in a 30 mo old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A 2 (HbA 2), absent 1 antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose 6 phosphate dehydrogenase, hexokinase, pyrivate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov Jacobs Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.

Original languageEnglish (US)
Pages (from-to)355-365
Number of pages11
JournalBlood
Volume49
Issue number3
StatePublished - 1977

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Fetal Hemoglobin
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Erythrocytes
Carbonic Anhydrase I
Population
Carbonic Anhydrase II
Hemoglobin A
Hexokinase
Glucosephosphate Dehydrogenase
L-Lactate Dehydrogenase
Hemolysis
Phosphotransferases
Cells
Antigens
Cell Count
Enzymes

ASJC Scopus subject areas

  • Hematology

Cite this

Changing erythrocyte populations in juvenile chronic myelocytic leukemia : evidence for disordered regulation. / Dover, George J; Boyer, S. H.; Zinkham, W. H.; Kazazian, Haig; Pinney, D. J.; Sigler, A.

In: Blood, Vol. 49, No. 3, 1977, p. 355-365.

Research output: Contribution to journalArticle

Dover, George J ; Boyer, S. H. ; Zinkham, W. H. ; Kazazian, Haig ; Pinney, D. J. ; Sigler, A. / Changing erythrocyte populations in juvenile chronic myelocytic leukemia : evidence for disordered regulation. In: Blood. 1977 ; Vol. 49, No. 3. pp. 355-365.
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abstract = "Fetal and adult erythrocyte characteristics were studied serially in a 30 mo old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69{\%} hemoglobin F (HbF), 1.1{\%} hemoglobin A 2 (HbA 2), absent 1 antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose 6 phosphate dehydrogenase, hexokinase, pyrivate kinase, and lactate dehydrogenase; 100{\%} of the erythrocytes present contained HbF. However, Orskov Jacobs Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17{\%}; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30{\%}. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.",
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AU - Sigler, A.

PY - 1977

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N2 - Fetal and adult erythrocyte characteristics were studied serially in a 30 mo old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A 2 (HbA 2), absent 1 antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose 6 phosphate dehydrogenase, hexokinase, pyrivate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov Jacobs Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.

AB - Fetal and adult erythrocyte characteristics were studied serially in a 30 mo old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A 2 (HbA 2), absent 1 antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose 6 phosphate dehydrogenase, hexokinase, pyrivate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov Jacobs Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.

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