Publication: Ultrastructural findings of congenital dyserythropoietic
sickle cell beta thal-associated anemia
Authors
Dell`Orbo, Carlo ; Marchi, Antonietta ; Quacci, Daniela
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
The ultrastructural findings of
erythroblasts and reticulocytes in one case of
congenital dyserythropoiethic anemia (CDA)
associated with a haemoglobinopathy, sickle cell beta
thalassemia minor (Type V CDA), is described.
The observations can be summarized as follows:
1) A lot of large breaks are present in the
erythroblast nuclear envelope.
2) Nuclear membrane evaginations are filled with
dense loose chromatin.
3) Electron-transparent areas (moth eaten
chromatin) are evident in dense chromatin.
4) Electron-dense granular material, related to
altered haemoglobin chain storage, is evident in the
nucleus and in the cytoplasm.
5) Iron deposits are present in mitochondrial
matrix.
6) Myelinic figures are present in reticulocyte
cytoplasm.
For the first time the ultrastructural findings in
this type of associated CDA are described and related
to the double origin of clinical symptoms.
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Citation
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