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The haematology of homozygous sickle cell disease after the age of 40 years
Author(s) -
Morris Joanne,
Dunn David,
Beckford Marjorie,
Grandison Yvonne,
Mason Karlene,
Higgs Douglas,
Ceulaer Karel De,
Serjeant Beryl,
Serjeant Graham
Publication year - 1991
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.1365-2141.1991.tb08588.x
Subject(s) - medicine , hematology , platelet , bone marrow , reticulocyte , disease , creatinine , red cell , gastroenterology , physiology , biology , biochemistry , messenger rna , gene
Summary Haematological indices have been studied in 181 patients with homozygous sickle cell (SS) disease aged 40–73 years. Cross‐sectional analyses in 5‐year age bands indicated age‐related decreases in HbF (males only), total haemoglobin and platelet counts. Longitudinal studies within individuals confirmed the downward age‐related trend in haemoglobin and platelets and also revealed a falling reticulocyte count, most significant when expressed as absolute values. Total nucleated cells also fell although the decline was significant only in females. These observations are consistent with a progressive bone marrow failure which is not explained by the commonly occurring renal impairment in older SS patients since the changes persisted in analyses confined to patients with normal creatinine levels. The mechanism of this bone marrow failure is currently unknown.

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