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Studies of Ineffective Erythropoiesis and Peripheral Haemolysis in Congenital Dyserythropoietic Anaemia Type II
Author(s) -
Barosi G.,
Cazzola M.,
Stefanelli M.,
Ascari E.
Publication year - 1979
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.1365-2141.1979.tb03747.x
Subject(s) - haemolysis , ineffective erythropoiesis , erythropoiesis , splenectomy , medicine , peripheral blood , peripheral , hemolysis , gastroenterology , immunology , anemia , spleen
S ummary . Erythrokinetic parameters were estimated in six patients suffering from congenital dyserythropoietic anaemia type II (CDA II) by means of a mathematical model of iron kinetics. A wide variation in effectiveness of erythroid activity was observed, and a significant negative correlation was found between ineffective erythropoiesis and peripheral haemolysis. In four patients with prominent peripheral haemolysis, splenectomy was carried out. Marked improvement in their clinical condition and in haemoglobin level resulted.