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 Haematoljournal_title
British journal of haematologyauthors
Steinberg MH,Coleman MB,Adams JG,Platica O,Gillette P,Rieder RFdoi
10.1111/j.1365-2141.1983.tb02164.xsubject
Has Abstractpub_date
1983-11-01 00:00:00pages
487-92issue
3eissn
0007-1048issn
1365-2141journal_volume
55pub_type
杂志文章abstract::The presence of autoimmune diseases, including Systemic Sclerosis (SSc), suggest failure of the normal immune regulatory processes leading to activation and expansion of autoreactive effector immune cells. Recently, stem cell transplantation emerged as a novel rescue therapy for a variety of refractory autoimmune dise...
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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doi:10.1111/j.1365-2141.1984.tb02190.x
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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doi:
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pub_type: 杂志文章
doi:10.1111/j.1365-2141.1978.tb07103.x
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pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
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