Relation between Erythropoiesis and Bone Metabolism in Thalassemia

Pootrakul, Pensri; Hungsprenges, Suwanna; Fucharoen, Suthat; Baylink, David J.; Thompson, E.; English, Eugenia; Lee, Minako; Burnell, James M.; Finch, Clement A.

doi:10.1056/nejm198106113042406

Cited by 78 publications

(24 citation statements)

References 17 publications

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“…With regard to that point we have to stress the different etiologies of bone loss between TM and TI. The erythroid marrow expansion and subsequent hyperactive marrow impairs bone production and is considered the major cause of osteoporosis in TI [2]. However, we found only weak correlation between baseline sTfR levels and lumbar spine BMD (r = –0.295, p = 0.06), while there was no correlation between EPO or hemolytic parameters (indirect bilirubin, reticulocytes counts and lactate dehydrogenase) with BMD of all studied sites or with CTX levels.…”

Section: Introductioncontrasting

confidence: 54%

“…Patients with TI seem to have a more pronounced marrow erythropoiesis than TM patients [1], which leads to expansion of medullary cavities, pressure on cortical bone and subsequently bone loss, pain and skeletal abnormalities in several cases [2]. Therefore, the pathogenesis of osteoporosis in TI is considered to be mainly due to ineffective hemopoiesis and progressive marrow expansion and seems to differ from the mechanisms of bone loss in TM that are very complicated and include several factors, such as endocrine dysfunction, iron overload, deferoxamine effect on osteoblasts, marrow expansion and genetic abnormalities [3, 4].…”

Section: Introductionmentioning

confidence: 99%

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Continuous Increase in Erythropoietic Activity despite the Improvement in Bone Mineral Density by Zoledronic Acid in Patients with Thalassemia Intermedia-Induced Osteoporosis

Voskaridou

Christoulas²,

Antoniadou³

et al. 2008

Acta Haematol

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Section: Introductioncontrasting

confidence: 54%

Section: Introductionmentioning

confidence: 99%

Continuous Increase in Erythropoietic Activity despite the Improvement in Bone Mineral Density by Zoledronic Acid in Patients with Thalassemia Intermedia-Induced Osteoporosis

Voskaridou

Christoulas²,

Antoniadou³

et al. 2008

Acta Haematol

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“…The vertebrae is an important site for marrow expansion and significant cortical thinning and disruption of trabecular bone can occur (66,110). In contrast, the relative absence of bony deformity, red marrow activity and overlying soft tissue artifact at the femoral neck enables a more accurate assessment of BMD using DXA in longitudinal analysis (111).…”

Section: E Bone Marrow Expansionmentioning

confidence: 99%

Bone Disease in Thalassemia: A Molecular and Clinical Overview

et al. 2016

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Thalassemia bone disease is a common and severe complication of thalassemia-an inherited blood disorder due to mutations in the α or β hemoglobin gene. In its more severe form, severe anemia is present, and treatment with frequent red blood cell transfusion is necessary. Because the body has limited capacity to excrete iron, concomitant iron chelation is required to prevent the complications of iron overload. The effects of chronic anemia and iron overload can lead to multiple end-organ complications such as cardiomyopathy, increased risks of blood-borne diseases, and liver, pituitary, and bone disease. However, our understanding of thalassemia bone disease is incomplete and is composed of a complex piecemeal of risk factors that include genetic factors, hormonal deficiency, marrow expansion, skeletal dysmorphism, iron toxicity, chelators, and increased bone turnover. The high prevalence of bone disease in transfusion-dependent thalassemia is seen in both younger and older patients as life expectancy continues to improve. Indeed, hypogonadism and GH deficiency contribute to a failure to achieve peak bone mass in this group. The contribution of kidney dysfunction to bone disease in thalassemia is a new and significant complication. This coincides with studies confirming an increase in kidney stones and associated accelerated bone loss in the thalassemia cohort. However, multiple factors are also associated with reduced bone mineral density and include marrow expansion, iron toxicity, iron chelators, increased bone turnover, GH deficiency, and hypogonadism. Thalassemia bone disease is a composite of not only multiple hormonal deficiencies but also multiorgan diseases. This review will address the molecular mechanisms and clinical risk factors associated with thalassemia bone disease and the clinical implications for monitoring and treating this disorder.

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“…Thalassaemia is a hereditary disease causing unbalanced globin chain synthesis, which results in ineffective erythropoiesis and increased peripheral haemolysis. Consequently, increased erythropoiesis as well as ineffective erythropoiesis in bone marrow results in expansion of bone marrow cavity and subsequently decreased cortical and trabecular bone tissues causing osteoporosis (Pootrakul et al ., 1981; Schettini et al ., 1987; Mohamed & Jackson, 1998; Soliman et al ., 1998). Moreover, iron deposit in bone (Bordat et al ., 1993), hypogonadism/delayed puberty (Finkelstein et al ., 1992; Anapliotou et al ., 1995) vitamin D deficiency (Aloia et al ., 1982; Dandoma et al ., 1987; Moulas et al ., 1997), GH/IGF‐1 deficiency (Low et al ., 1998; Soliman et al ., 1998) and desferrioxamine‐induced bony dysplasia may act in concert to reduced bone mass (De Virgilis et al ., 1988; Brill et al ., 1991; Olivieri et al ., 1992).…”

mentioning

confidence: 99%