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Thalassemia major, a disease characterized not only by markedly shortened red blood cell survival and ineffective erythropoiesis necessitating numerous transfusions throughout life, but also by excessive absorption of iron from the gastrointestinal tract, leads to diffuse iron storage in both the reticuloendothelial system and the parenchyma itself. It is the very early onset of massive iron deposition, combined with the clinical manifestations linked with it, that sets this disease apart from simple transfusion siderosis and also from idiopathic hemochromatosis.
All 4 patients studied received extraneous iron by transfusion totaling 50-150 gm. In the 2 patients studied who were autopsied at The New York Hospital, all lymph nodes contained variable amounts of iron. The most severely involved lymph nodes were present in the chest, abdomen and along the vertebral column. In these locations their iron content approximated 24 per cent of their dry weight. The calcium content was only 0.27 per cent of dry weight. Therefore, the radiodensity of the lymph nodes is caused by iron storage and not dystrophic calcification.
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