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Erythropoietin production and erythropoiesis in compensated and anaemic states of hereditary spherocytosis
Author(s) -
Guar Roberta,
Centenara Esther,
Zappa Manuela,
Zanella Alberto,
Barosi Giovanni
Publication year - 1996
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1996.00285.x
Subject(s) - erythropoiesis , erythropoietin , hereditary spherocytosis , medicine , erythropoietin receptor , anemia , endocrinology , ineffective erythropoiesis , spherocytosis , iron deficiency , splenectomy , spleen
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%) had 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 ln 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.