Premium
The Ultrastructure of Erythropoiesis in Two Haemoglobinopathies
Author(s) -
Frisch B.,
Lewis S. M.,
Sherman D.,
White J. M.,
GordonSmith E. C.
Publication year - 1974
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1974.tb06644.x
Subject(s) - ultrastructure , cytoplasm , erythropoiesis , endoplasmic reticulum , multinucleate , ineffective erythropoiesis , pathology , microtubule , microbiology and biotechnology , bone marrow , biology , mitochondrion , nuclear membrane , chemistry , medicine , anemia
S ummary . The ultrastructural features of the bone marrow have been studied in two patients with haemoglobinopathy, Hb Hammersmith and Hb Nottingham respectively. Multiple anomalies were observed in erythroblasts in both cases, together with islands of normal erythropoiesis. The incidence of abnormalities was higher in the case of Hb Nottingham, but many of the features seen were similar in the two cases. The findings included bi‐ and multinucleated erythroblasts, cytoplasmic bridges and microtubules, abnormalities of the nuclear envelope, widened nuclear pores, alterations in the structure and density of the chromatin, abundant iron‐laden mitochondria, a variety of nuclear and cytoplasmic inclusions, and the presence within the cytoplasm of membranous material which is presumed to be endoplasmic reticulum. Many of these ultrastructural alterations are similar to those which have been observed in the congenital dyserythropoietic anaemias. A particular feature of the present cases was localized myelinization in both nuclear envelope and the membranes of the endoplasmic reticulum. The implications of the findings are discussed in relation to known cellular responses to various stimuli, including injury. It is concluded that the wide range of ultrastructural aberrations which occur in the erythroblasts are not specific to the haemoglobinopathies or the congenital dyserythropoietic anaemias, but are indicative of cellular injury, irrespective of the fundamental aetiology.