Red cell production and destruction in haemoglobin H disease

M. Cazzola, G. Barosi, E. Orlandi, G. Santagati

Research output: Contribution to journalArticle

Abstract

The authors have studied erythropoiesis in a woman with H hemoglobin disease. In particular they evaluated quantitatively the pathogenetic mechanisms of the anemia. The results were compared with those obtained in 4 patients with intermediate beta thalassemia that has an anemia comparable to that of the woman patient with alpha thalassemia. Both the inefficacious erythropoiesis and the peripheral hemolysis are found to be important factors in the pathogenesis of the anemia of the woman patient with hemoglobin H disease. With respect to the 4 cases of intermediate beta thalassemia, however, there was a lesser grade of inefficacious erythropoiesis and a more marked peripheral hyperhemolysis. These results confirm the hypothesis that the tetramers of the beta chain, that constitute the H hemoglobin, tend to precipitate more in the circulating erythrocytes than in the medullary erythrocytes.

Original languageEnglish
Pages (from-to)780-784
Number of pages5
JournalHaematologica
Volume65
Issue number6
Publication statusPublished - 1980

ASJC Scopus subject areas

  • Hematology

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