Erythropoietin production and erythropoiesis in compensated and anaemic states of hereditary spherocytosis

Roberta Guarnone, Esther Centenara, Manuela Zappa, Alberto Zanella, Giovanni Barosi

Research output: Contribution to journalArticlepeer-review


A compensated haemolytic state is defined by decreased red cell life-span without anaemia, i.e. by increased erythropoiesis in the absence of the physiological stimulus for erythropoietin (Epo) production. We evaluated s-Epo levels and the expansion of erythropoiesis (as measured by circulating transferrin receptor, s-TfR) in 32 patients with hereditary spherocytosis (HS) with the aim of verifying whether the enhanced erythropoiesis of compensated haemolysis was Epo-dependent. 20 of the patients (62.5%) bad normal Hb values (> 12 g/dl in females and > 13 g/dl in males). Their compensated haemolytic state was the result of up to 8.2 times normal s-Epo and up to 3.9 times normal s-TfR levels, which were maintained by physiological regulation of erythropoiesis, as documented by the inverse dependence of Hb on s-Epo levels. Considering that patients with iron-deficiency anaemia represented the predicted physiological Epo response to anaemia, the observed/ predicted In s-Epo ratio (O/P ratio) was calculated in HS patients with anaemia and was used as an index of the adequateness of Epo production. All the anaemic HS patients had an O/P ratio > 1, documenting inappropriately high s-Epo levels. This work demonstrates that the compensated haemolytic state of HS patients is produced by an inappropriately high s-Epo level, and that the pattern of Epo overproduction is a biological characteristic of the disease.

Original languageEnglish
Pages (from-to)150-154
Number of pages5
JournalBritish Journal of Haematology
Issue number1
Publication statusPublished - 1996


  • Compensated haemolytic state
  • Erythropoiesis
  • Hereditary spherocytosis
  • Serum erythropoietin
  • Serum transferrin receptor

ASJC Scopus subject areas

  • Hematology


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