A Comparative Electron Microscopic Study of Refractory and Alcoholic Sideroblastic Anaemia

Grasso, Joseph A.; Hines, John

doi:10.1111/j.1365-2141.1969.tb05662.x

Cited by 38 publications

(4 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The mitochondrial abnormalities encountered in the erythropoietic cells of the present cases were identical to those previously described in other cases of primary acquired sideroblastic anaemia (Bessis & Breton-Gorius, 1962;Sorenson, 1962;Larizza & Orlandi, 1964;Petz et at, 1966;Grasso & Hines, 1969). Our patients also showed one or more other ultrastructural x 7500.…”

Section: Discussionsupporting

confidence: 89%

Capacity of Ringed Sideroblasts to Synthesize Nucleic Acids and Protein in Patients with Primary Acquired Sideroblastic Anaemia

Wickramasinghe

Hughes

1978

Br J Haematol

View full text Add to dashboard Cite

Bone marrow cells from two patients with primary acquired sideroblastic anaemia were studied using the technique of electron microscope autoradiography. Whereas a significant proportion of erythroblasts containing small to moderate quantities of intramitochondrial deposits incorporated [3H]thymidine into DNA, the majority of cells containing large quantities of intramitochondrial deposits did not. These results are consistent with the idea that several of the early polychromatic cells with large quantities of deposits become arrested in their progress through the cell cycle. The data also revealed that the accumulation of increasing quantities of iron-containing material within the mitochondria of an erythroblast was frequently (but not invariably) associated with and was possibly responsible for (I) a depression of RNA synthesis and (2) a more marked depression of protein synthesis. A small proportion of the erythroblasts showing a depression of protein synthesis also displayed various non-specific ultrastructural abnormalities indicative of cellular injury such as the presence of intranuclear clefts, nuclear membrane-associated myelin figures and lipid-laden intracytoplasmic vacuoles.

show abstract

Section: Discussionsupporting

confidence: 89%

Capacity of Ringed Sideroblasts to Synthesize Nucleic Acids and Protein in Patients with Primary Acquired Sideroblastic Anaemia

Wickramasinghe

Hughes

1978

Br J Haematol

View full text Add to dashboard Cite

show abstract

“…The syndrome is characterized morphologically by ring sideroblasts, which are erythroblasts containing mitochondria laden with nonferritin iron particles surrounding the nucleus. Electron microscopy of these cells demonstrates abnormally increased amounts of amorphous nonferritin iron deposited between the cristae of the mitochondria, which are swollen and deformed [4].…”

Section: Introductionmentioning

confidence: 99%

Increased bone marrow delta‐aminolevulinic acid synthetase activity in the acute reversible sideroblastic anemia of alcoholics

Fraser

Schacter

1980

American J Hematol

View full text Add to dashboard Cite

In an effort to clarify the biochemical pathogenesis of the acute reversible siderblastic anemia of alcoholics, bone marrow delta-aminolevulinic acid (ALA) synthetase activity was measured in anemic alcoholic patients with and without pathological ring sideroblasts in their marrows and compared with the activity of this enzyme in normal subjects and in patients with idiopathic sideroblastic anemia. Mean ALA-synthetase activity measured (following addition of exogenous pyridoxal phosphate in three patients with the ring sideroblast morphologic abnormality) was 502 f 147 (SE) pmoles ALA per lo6 erythroblasts per 30 minutes, as compared with 201 f 69 in the seven alcoholic patients without pathologic sideroblastic marrow abnormalities, 169 f 32 in the six normal subjects, and 52 * 4 pmoles ALA per lo6 erythroblasts per 30 minutes in two patients with idiopathic sideroblastic anemia. Comparison of enzyme activity in the presence and absence of pyridoxal phosphate revealed that addition of this enzyme cofactor accentuated ALA-synthetase activity by an average of only 24% in the alcoholic sideroblastic group, as compared with 42% accentuation in the normal group and 8% accentuation in the idiopathic sideroblastic group. These results suggest that abnormalities in pyridoxal phosphate metabolism in alcoholics may not be of importance in the inhibition of heme biosynthesis at the level of ALA synthetase but, rather, that an enzymatic or metabolic block should be sought at a more distal site in the heme biosynthetic pathway.

show abstract

“…However, as the proportion of cells in G-z was highest in those early polychromatic erythroblasts with the largest accumulations of stainable iron and because only a small proportion of early polychromatic ring sideroblasts were found to be in DNA synthesis, the abnormalities were attributed to an arrest of proliferation caused by the pathological accumu lation of iron within these cells. In support of this hypothesis was the electron microscopic demonstration of iron-laden mitochondria in the early and late erythroblasts in this disease [6,11], and the fact that mitochondrial damage is known to produce a G2 arrest [4]. In the 3 cases of hereditary sideroblastic anaemia, there was no 'pile up' of basophilic or early polychromatic erythroblasts in G> and only a small number of 'U' cells were present.…”

Section: Discussionmentioning

confidence: 99%

Microspectrophotometric and Electron Microscopic Studies of Bone Marrow in Hereditary Sideroblastic Anaemia

Wickramasinghe¹,

Fulker²,

Losowsky³

et al. 1971

Acta Haematol

View full text Add to dashboard Cite

Ultrastructural studies in 3 patients with hereditary sideroblastic anaemia have shown iron-laden mitochondria in a proportion of the non-dividing, late polychromatic erythroblasts. In contrast to the situation in primary acquired sideroblastic anaemia, the mitochondria of the proliferating erythroblasts were rarely infiltrated with iron; and a combined quantitative cytochemical and autoradiographic study showed no significant arrest of cell proliferation. It is likely that impaired haemoglobin synthesis is more important than ineffective erythropoiesis in the pathogenesis of theanaemia in this disease

show abstract

A Comparative Electron Microscopic Study of Refractory and Alcoholic Sideroblastic Anaemia

Cited by 38 publications

References 23 publications

Capacity of Ringed Sideroblasts to Synthesize Nucleic Acids and Protein in Patients with Primary Acquired Sideroblastic Anaemia

Capacity of Ringed Sideroblasts to Synthesize Nucleic Acids and Protein in Patients with Primary Acquired Sideroblastic Anaemia

Increased bone marrow delta‐aminolevulinic acid synthetase activity in the acute reversible sideroblastic anemia of alcoholics

Microspectrophotometric and Electron Microscopic Studies of Bone Marrow in Hereditary Sideroblastic Anaemia

Contact Info

Product

Resources

About