The effect of erythroid hyperplasia on iron balance

Pootrakul, Pensri; Kitcharoen, Kriengkrai; Yansukon, Pornpan; Wasi, P; Fucharoen, Suthat; Charoenlarp, P; Brittenham, Gary M.; Pippard, M. J.; Finch, CA

doi:10.1182/blood.v71.4.1124.bloodjournal7141124

Cited by 35 publications

(43 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In addition, splenectomized thalassemia Thai patients do not receive regular transfusions, although they are severely afflicted patients as measured by their iron loading status (19). Splenectomized β‐thalassemia/Hb E patients are severely iron‐overloaded due to iron absorption in response to ineffective erythropoiesis (18). Therefore, iron overload, both from increased iron absorption or chronic blood transfusions, may affect the regulation and redistribution of lymphocyte subsets from the spleen and lymph nodes to the circulating pool and vice versa.…”

Section: Discussionmentioning

confidence: 99%

Lymphocyte subsets and specific T-cell immune response in thalassemia

et al. 2000

View full text Add to dashboard Cite

Infection is very common in thalassemia and is one of the major causes of death. To date, it is not quite clear why these patients are susceptible to infection. In this study, lymphocyte immunophenotyping for CD3+ (T‐cells), CD3+CD4+ (T‐helper/inducer cells), CD3+CD8+ (T‐suppressor/cytotoxic cells), CD3−CD19+ (B‐cells), and CD3−CD16/56+ (natural killer cells) subsets and expression of the activation antigen CD69 on CD3+CD4+ and CD3+CD8+ T‐cells were determined in the whole blood of thalassemia patients, using a three‐color flow cytometric technique. Results showed that only splenectomized β‐thalassemia/hemoglobin (Hb) E patients displayed a marked increase in absolute number of all lymphocytes. In addition, splenectomized β‐thalassemia/Hb E showed a significantly lower percentage of CD3+ cells, with a corresponding increase in CD19+ cells. These differences, when compared with normal subjects and other thalassemia patients, may be attributed to splenectomy. α‐thalassemia patients, on the other hand, showed no significant difference from the normal group. While lymphocyte subsets in splenectomized β‐thalassemia/Hb E patients showed an abnormal distribution, T‐cell activation in these patients was not different from the activation seen in normal subjects. This implies that thalassemia patients, during the steady state of disease, appear to have normal T‐lymphocyte function with only moderate abnormalities of T‐ and B‐lymphocyte subsets.Cytometry (Comm. Clin. Cytometry) 42:11–17, 2000. © 2000 Wiley‐Liss, Inc.

show abstract

Section: Discussionmentioning

confidence: 99%

Lymphocyte subsets and specific T-cell immune response in thalassemia

et al. 2000

View full text Add to dashboard Cite

show abstract

“…NTDT patients are susceptible to iron overload, although the mechanism of iron accumulation is quite different from that observed in β‐thalassemia major patients . Whilst NTDT patients receive no or only occasional transfusions, their intestinal iron absorption is continuously upregulated, leading to slow accumulation of iron in tissues, particularly in the liver . The mechanism of increased intestinal iron absorption in NTDT patients is triggered by a cascade initiated by ineffective erythropoiesis, which is characteristic of these diseases .…”

Section: Introductionmentioning

confidence: 99%

Treating iron overload in patients with non‐transfusion‐dependent thalassemia

et al. 2013

View full text Add to dashboard Cite

Despite receiving no or only occasional blood transfusions, patients with non-transfusion-dependent thalassemia (NTDT) have increased intestinal iron absorption and can accumulate iron to levels comparable with transfusion-dependent patients. This iron accumulation occurs more slowly in NTDT patients compared to transfusion-dependent thalassemia patients, and complications do not arise until later in life. It remains crucial for these patients' health to monitor and appropriately treat their iron burden. Based on recent data, including a randomized clinical trial on iron chelation in NTDT, a simple iron chelation treatment algorithm is presented to assist physicians with monitoring iron burden and initiating chelation therapy in this group of patients. Am. J. Hematol. 88:409–415, 2013. © 2013 Wiley Periodicals, Inc.

show abstract

“…Patients with β ‐thalassaemia major (TM) exhibit a reduction or a lack of synthesis of the β ‐chain of haemoglobin (Weatherall et al , 1981), while the remaining excess α ‐chains form unstable aggregates that result in premature destruction of the erythrocyte. This leads to severe anaemia, increased erythrocyte turnover and ineffective erythropoiesis (Pootrakul et al , 1988; Rund & Rachmilewitz, 2005). To treat the anaemia, patients regularly undergo blood transfusions, which exacerbate their iron overload caused by increased intestinal absorption.…”

mentioning

confidence: 99%

Downregulation of hepcidin and haemojuvelin expression in the hepatocyte cell‐line HepG2 induced by thalassaemic sera

et al. 2006

View full text Add to dashboard Cite

Summary β‐Thalassaemia represents a group of diseases, in which ineffective erythropoiesis is accompanied by iron overload. In a mouse model of β‐thalassaemia, we observed that the liver expressed relatively low levels of hepcidin, which is a key factor in the regulation of iron absorption by the gut and of iron recycling by the reticuloendothelial system. It was hypothesised that, despite the overt iron overload, a putative plasma factor found in β‐thalassaemia might suppress liver hepcidin expression. Sera from β‐thalassaemia and haemochromatosis (C282Y mutation) patients were compared with those of healthy individuals regarding their capacity to induce changes the expression of key genes of iron metabolism in human HepG2 hepatoma cells. Sera from β‐thalassaemia major patients induced a major decrease in hepcidin (HAMP) and lipocalin2 (oncogene 24p3) (LCN2) expression, as well as a moderate decrease in haemojuvelin (HFE2) expression, compared with sera from healthy individuals. A significant correlation was found between the degree of downregulation of HAMP and HFE2 induced by β‐thalassaemia major sera (r = 0·852, P < 0·0009). Decreased HAMP expression was also found in HepG2 cells treated with sera from β‐thalassaemia intermedia patients. In contrast, the majority of sera from hereditary haemochromatosis patients induced an increase in HAMP expression, which correlated with transferrin (Tf) saturation (r = 0·765, P < 0·0099). Our results suggest that, in β‐thalassaemia, serum factors might override the potential effect of iron overload on HAMP expression, thereby providing an explanation for the failure to arrest excessive intestinal iron absorption in these patients.

show abstract

The effect of erythroid hyperplasia on iron balance

Cited by 35 publications

References 0 publications

Lymphocyte subsets and specific T-cell immune response in thalassemia

Lymphocyte subsets and specific T-cell immune response in thalassemia

Treating iron overload in patients with non‐transfusion‐dependent thalassemia

Downregulation of hepcidin and haemojuvelin expression in the hepatocyte cell‐line HepG2 induced by thalassaemic sera

Contact Info

Product

Resources

About