The effects of alpha-thalassaemia in HbSC disease.

Abstract:

:In HbSC disease, as in sickle cell anaemia, there is a spectrum of clinical severity. A reduced mean corpuscular volume and haemoglobin concentration, traits typical of thalassaemia, might retard sickling. We therefore ascertained the prevalence of alpha-thalassaemia in 53 adults with HbSC disease and related alpha-globin gene deletion to the haematologic and clinical findings. Alpha-globin genotype was identified by restriction endonuclease gene mapping. Indirect ophthalmoscopy and fluorescein angiography were used to document the presence of proliferative retinopathy. Bone necrosis and infarction were determined roentgenographically or by radionuclide scanning. Either heterozygous or homozygous alpha-thalassaemia-2 was present in 26% of patients. There was no relationship between alpha-globin genotype and haematocrit, pain crises, bone lesions, proliferation retinopathy or clinical severity score.

journal_name

Br J Haematol

authors

Steinberg MH,Coleman MB,Adams JG,Platica O,Gillette P,Rieder RF

doi

10.1111/j.1365-2141.1983.tb02164.x

subject

Has Abstract

pub_date

1983-11-01 00:00:00

pages

487-92

issue

3

eissn

0007-1048

issn

1365-2141

journal_volume

55

pub_type

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