Premium
Red cell kinetics in thalassaemia intermedia: its use for a prospective prognosis
Author(s) -
Najean Y.,
Deschryver F.,
Henni T.,
Girot R.
Publication year - 1985
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 - 10.1111/j.1365-2141.1985.tb07340.x
Subject(s) - ineffective erythropoiesis , medicine , gastroenterology , splenectomy , haemolysis , thalassemia , bone marrow , erythropoiesis , red cell , beta thalassemia , red blood cell , anemia , spleen , immunology
S ummary A kinetic study of erythroid cell production and destruction (radioactive iron incorporation, red blood cell survival time, bone marrow scintigraphy) was performed in 12 cases of thalassaemia intermedia (six adults and six children), classified retrospectively, and compared to that performed in 17 cases of Cooley's anaemia. Our results confirm the genetic heterogeneity of these cases, the level of Hb F varies from 30% to 100% and the non α/α chain synthesis is only statistically superior to that seen in Cooley's disease. On the other hand, the kinetic study clearly separates the cases who are, or will be, clinically intermediate, showing a higher radioactive iron medullary uptake, a less ineffective erythropoiesis than that seen in Cooley's disease, and a greater peripheral haemolysis. In our study, no overlap was seen between the two groups. Iron kinetic study is then of prognostic interest and may help in therapy decisions, transfusion regimen and iron chelation, and splenectomy.