Abstract:
:The contribution of red cell size (mean cell volume) to the clinical and haematological manifestations of homozygous sickle cell (SS) disease has been investigated by comparing the features of two groups of patients with low (less than or equal to 80 fl) and high (greater than 95 fl) MCV values after matching for age, sex and fetal haemoglobin level. The microcytic group manifested significantly higher Hb, PCV, RBC and HbA2 levels and significantly lower reticulocyte and irreversibly sickled-cell counts. Clinical features were not less severe in the microcytic group, splenomegaly persisting for longer and painful crises were more common although the latter difference did not reach significance. The milder haematological picture associated with decreased intravascular sickling was not reflected in a more mild clinical course. It is postulated that the higher viscosity accompanying the higher haemoglobin levels in microcytic patients may offset the rheological advantages of decreased intravascular sickling.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Serjeant GR,Foster K,Serjeant BEdoi
10.1111/j.1365-2141.1981.tb02736.xsubject
Has Abstractpub_date
1981-07-01 00:00:00pages
445-9issue
3eissn
0007-1048issn
1365-2141journal_volume
48pub_type
杂志文章abstract::Establishing a precise diagnosis is essential in inborn haematological cytopenias to enable appropriate treatment decisions and avoid secondary organ damage. However, both diversity and phenotypic overlap of distinct disease entities may make the identification of underlying genetic aetiologies by classical Sanger seq...
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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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pub_type: 杂志文章
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更新日期:1980-04-01 00:00:00
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pub_type: 临床试验,杂志文章
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pub_type: 临床试验,杂志文章
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pub_type: 杂志文章,评审
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更新日期:2015-09-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
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更新日期:1979-01-01 00:00:00
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journal_title:British journal of haematology
pub_type: 杂志文章
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更新日期:1999-08-01 00:00:00