An unusual case of leukemia with high fetal hemoglobin: demonstration of abnormal hemoglobin synthesis localized in a red cell clone

Content Provider	Scilit
Author	Pagnier, J. Lopez, M. Mathiot, C. Habibi, B. Zamet, P. Varet, B. Labie, D.
Copyright Year	1977
Description	A high level of fetal hemoglobin was found in an 8-yr-old boy without any hematologic disorders except for a moderate anemia. The absence of hemoglobin abnormalities in the parents led us to suspect a latent malignant disease that, on follow-up, was confirmed to be myelomonocytic leukemia. Hemoglobin biosynthetic studies provided evidence of unbalanced synthesis of globin subunits by reticulocytes, while the production of non-alpha chains was equal to that of alpha chains in bone marrow cells. The expression of red cell antigen i was increased, while those of I, A, and A1 antigens were found to decrease progressively. Two populations of erythrocytes, A-positive and A- negative, were distinguished and could be separated by differential agglutination. Unbalanced globin chain synthesis, increased fetal hemoglobin, and antigenic changes of the membrane were shown to be restricted to the A-negative population. The biologic data were not entirely consistent with a genuine reversion to fetal erythropoiesis. The question remains of a polychromosomal lesion of either quiescent F cells or adult stem cells.
Related Links	https://ashpublications.org/blood/article-pdf/50/2/249/204527/249.pdf
Ending Page	258
Page Count	10
Starting Page	249
DOI	10.1182/blood.v50.2.249.bloodjournal502249
Journal	Blood
Issue Number	2
Volume Number	50
Language	English
Publisher	American Society of Hematology
Publisher Date	1977-08-01
Access Restriction	Open
Subject Keyword	Pediatrics and Child Health Antigenic Bone Marrow Fetal Hemoglobin Abnormal Alpha Differential Adult Unbalanced Journal: Blood (Vol- 47, Issue- 2)
Content Type	Text
Resource Type	Article