UNUSUAL CASE OF LEUKEMIA WITH HIGH FETAL HEMOGLOBIN - DEMONSTRATION OF ABNORMAL HEMOGLOBIN SYNTHESIS LOCALIZED IN A RED-CELL CLONE

  • 1 January 1977
    • journal article
    • research article
    • Vol. 50  (2) , 249-258
Abstract
A high level of fetal Hb was found in an 8 yr old boy without any hematologic disorders except for a moderate anemia. The absence of Hb abnormalities in the parents led to suspicion of a latent malignant disease that, on follow-up, was confirmed to be myelomonocytic leukemia. Hb 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 decreased 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 Hb and antigenic changes of the membrane were 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 quiescent F cells or adult stem cells.

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