Use of the ferritin/alanine aspartate transaminase ratio as an iron overload marker independent of liver cell damage.

To define an iron overload index independent of liver cell damage, the mean annual levels of alanine aspartate transaminase (ALAT) and serum ferritin and their ratios were determined. Ferritin/ALAT ratio values were compared between two groups of patients with acute or chronic hepatitis without iron overload, and one group of thalassaemic patients with iron overload. The two groups without iron overload exhibited ferritin/ALAT ratio values of 2 and 1.2 respectively; a ratio value higher than 10 was always observed in those patients with iron overload. The ferritin/ALAT ratio is correlated with the degree of iron overload. This ratio increases in regularly-transfused patients without chelation treatment. It generally remains stable or decreases after initiation of iron chelation therapy. The ferritin/ALAT ratio thus appears useful in the follow-up of patients subjected to a long-term transfusional treatment particularly when acute or chronic liver cell damage may interfere with iron overload by increasing serum ferritin values.

Neocytopheresis: a new approach for the transfusion of patients with thalassaemia major.

At present the treatment of thalassaemia major consists of regular blood transfusions coupled with chelation therapy using deferoxamine. A complementary approach to the problem is the use of blood units enriched with young red cells (neocytes), which reduce the transfusional frequency and thereby diminish the risk of iron overload. Young red cell units were collected from blood from 60 volunteer donors using a cell separator (IBM 2997). Donors' blood was anticoagulated and the young red cell harvesting carried out over 4 h at a constant rotor speed of 500 rpm. Three biological criteria were used to evaluate young red cell quality: the number of reticulocytes, the pyruvate kinase activity and the mean corpuscular volume, all of which show an enrichment of young red cells as compared to standard donor units. The 51Cr young red cell survival in four normal donors and in two splenectomized patients showed an increased red cell half-life compared to the same study performed with standard blood units. Blood consumption was diminished significantly when the two patients were transfused with young red cell units. It must be emphasized that, despite the high cost of this blood product, the efficiency of this transfusion technique, by reducing blood consumption, represents important progress and a hopeful treatment for chronic anaemia.

[Monitoring and appraisal of the effects of blood transfusion and iron chelation in thalassemia major]. / Surveillance et estimation des effets de la transfusion sanguine et de la chélation du fer dans la thalassémie majeure.

A total of 21 patients with thalassaemia major between 2 and 20-years-old were on a "hypertransfusion" regimen for 1 to 14 year periods. The use of the transfusion quotient (TQ) allowed a precise survey of the blood requirement. In all splenectomized patients the TQ was between 1 and 2; TQ increased and was always greater than 2 when patients became hypersplenic. Of the 21 regularly transfused patients, 12 had iron chelation therapy by continuous subcutaneous desferrioxamine (DF) injection (1 g over 8 to 12 h at home, nightly). The iron balance was negative for 7 patients, equilibrated for 1 and positive for 4 patients. All patients receiving more than injections of DF weekly had a negative balance; the variation of the serum ferritin level correlated with the iron balance (r = 0,77; P less than 0,01). The beneficial effects of the treatment were improvement in social and professional activity, in growth and puberty development and attenuation of the skull and face dysmorphia. Monitoring ferritin is a simple way to estimating the effects of chelation on iron overload.