Ineffective erythropoiesis in haemoglobin E beta -thalassaemia: an electron microscope study.
Electron microscope studies have been performed on the bone marrow cells of two non-splenectomized patients and the circulating erythroblasts and reticulocytes of three splenectomized patients with HbE/beta-thalassaemia. Some intracellular precipitates (probably consisting of alpha-chains) and mild dyserythropoietic changes were found in the early polychromatic erythroblasts within the bone marrow. Larger quantities of precipitate and more marked dyserythropoietic changes were found in the late polychromatic erythroblasts and reticulocytes both within the marrow and within the circulation. The bone marrow macrophages contained phagocytosed erythroblasts within their cytoplasm. These data indicate that the anaemia in HbE/beta-thalassaemia results largely from dyserythropoiesis and ineffective erythropoiesis. The ultrastructural abnormalities encountered in the cases of HbE/beta-thalassaemia were qualitatively and quantitatively similar to those seen in homozygous beta-thalassaemia.
D000328:Adult; D001853:Bone Marrow / Q000648:ultrastructure; D002648:Child; D004900:Erythroblasts / Q000648:ultrastructure*; D004912:Erythrocytes / Q000648:ultrastructure*; D004920:Erythropoiesis; D005260:Female; D006446:Hemoglobin E; D006801:Humans; D008264:Macrophages / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D012156:Reticulocytes / Q000648:ultrastructure; D013789:Thalassemia / Q000097:blood* / Q000473:pathology
D006446:Hemoglobin E