Abstract

A 17 year old Japanese girl is described with morphologic evidence of thalassemia characterized by an increase in fetal hemoglobin distributed heterogenously among the red cell population, a slight relative depression of α-chain synthesis, and an increase in γ-chain synthesis, as well as a significant depression in carbonic anhydrase activity.

The data suggest that some increase in the severity of these abnormalities took place with the development of a malignant thymoma. The combination of defects present here is not characteristic of the usual manifestations of α-thalassemia and might therefore be considered as an unusual variant of this condition.

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