Yuan et al., "Programmed cell death (PCD) and ineffective erythropoiesis
in Cooley's anemia," Blood, vol.
in beta-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin.
 Tumor necrosis factor-alpha has also been implicated and may cause ineffective erythropoiesis
The consequences of ineffective erythropoiesis
on hepcidin expression thus appear to trump the consequences of excessive iron stores.
in beta thalassemia is characterized by increased iron absorption mediated by down regulation of hepcidin and up regulation of ferroportin.
The inability to produce AY-globin chains allows the a-globin chains to accumulate and precipitate within erythroid precursors in the bone marrow.4 Typically, AY-thalassaemia is characterised by moderate to severe anaemia, caused by haemolysis and ineffective erythropoiesis
. The other signs, frequently observed in thalassaemic patients, include skeletal and/or endocrine changes and spleenomegaly, diarrhoea, irritability, fever, feeding problems and gradual bulging of the abdomen due to spleen and liver enlargement.5
When anemia is accompanied by increased erythroid activity and/or ineffective erythropoiesis
, e.g., in case of thalassemia and sideroblastic anemia, congenital dyserythropoietic anemia, and some myelodysplastic disorders, there is an appropriately increased absorption of iron from the diet because of higher needs of iron for hemoglobin synthesis (20,21).
The major factors causing black pigment stones are haemolytic anaemias, ineffective erythropoiesis
and increased production of bilirubin (caused by hereditary spherocytosis, thalassaemia, sickle cell disease, liver cirrhosis, malaria and ineffective erythropoiesis
This leads to excessive accumulation of alpha chains, which precipitate and cause lipid peroxidation of red cell membrane leading to haemolysis, ineffective erythropoiesis
and severe anaemia.1
Transferrin receptor concentrations are increased in disorders with expanded erythropoiesis, such as hemolytic anemias [15, 16, 18], and in conditions associated with ineffective erythropoiesis
, such as myelodysplastic syndromes and megaloblastic anemias [21, 22].
 and maintaining the circulating level of haemoglobin (Hb) sufficient to suppress endogenous ineffective erythropoiesis
Expansion of haemopoietic marrow due to ineffective erythropoiesis
causes mechanical interruption of bone formation and results in cortical thinning and fractures, as are also obvious in the radiographs of our patient (Figures-1 and 2).