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Title: Number and maturation of reticulocytes in various genotypes of thalassaemia as assessed by flow cytometry
Authors: Archrob Khuhapinant
Ahnond Bunyaratvej
Noriyuki Tatsumi
Montana Pribwai
Suthat Fucharoen
Department of Pathobiology
Faculty of Medicine, Ramathibodi Hospital, Mahidol University
Mahidol University
Osaka City University Medical School
Keywords: Medicine
Issue Date: 1-Jan-1987
Citation: Acta Haematologica. Vol.91, No.3 (1987), 119-125
Abstract: Ineffective erythropoiesis is a prominent defect leading to anaemic status in thalassaemic patients. Reticulocyte enumeration in the peripheral blood is a non-aggressive method of measuring bone marrow erythropoietic activity. We used an automated reticulocyte counter (Sysmex R-3000) to determine the number and maturation level of circulating reticulocytes among various types of thalassaemia: non-splenectomized β-thalassaemia/haemoglobin E (βE) and splenectomized cases (βE-S), classical haemoglobin H disease (H), haemoglobin H disease with haemoglobin Constant Spring (H/CS), homozygous haemoglobin Constant Spring (CS/CS), homozygous haemoglobin E (EE), heterozygous thalassaemics and other rare combinations. Haemoglobin H disease has a higher absolute count than β-thalassaemia (βE), indicating relatively better compensatory erythropoiesis in haemoglobin H disease. Those with CS genes (H/CS and CS/CS) have poorer reticulocyte maturation than any other type of thalassaemia with rather high absolute numbers, especially in H/CS. This indicates a severer degree of ineffective erythropoiesis in β-thalassaemia (ßE), which reflects an insufficient rise in reticulocyte number in comparison with α-thalassaemia (H). The presence of haemoglobin Constant Spring is associated with abnormally low reticulocyte maturation due to enhanced erythrocyte production or direct effect of Constant Spring globin itself, both still unexplained with the current information. The splenectomized βE has increased reticulocyte number and cells with high DNA content, probably nucleated red cells, designated as the upper particle count parameter. However, there is the same degree of reticulocyte maturation in non-splenecto- mized and splenectomized ßE patients, suggesting a role for splenic pooling of reticulocytes. © 1994 S. Karger AG, Basel.
ISSN: 14219662
Appears in Collections:Scopus 1969-1990

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