Electron Microscope and High Resolution Autoradiographic Studies of the Erythroblasts in Haemoglobin H Disease

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Abstract
EM studies of bone marrow from a patient with HbH disease revealed the presence of highly condensed branching intracytoplasmic inclusions within a proportion of the early and late polychromatic erythroblasts and reticulocytes. Intraerythroblastic inclusions of this type have not yet been described in any other hematological disorder and may be a specific feature of HbH disease. EM autoradiographic studies of the marrow cells from the patient with HbH disease revealed that active protein synthesis continues in inclusion-containing erythroblasts and there is a rapid precipitation of a considerable proportion of the newly synthesized free .beta. chains onto the surfaces of preformed inclusions, presumably after conversion to HbH. These EM and EM autoradiographic findings are quite different from the findings previously reported in homozygous .beta. thalassemia using the same techniques.