Abstract:
:Auto-immune haemolytic anaemia (AIHA) is characterised by haemolysis associated with the presence of the immunoglobulins IgG, IgM or IgA, and/or components of the complement system on the red cell membrane. The immunoglobulins react as auto-antibodies against the red cell antigens of the patient. IgG antibodies and the complement component C3d can be detected by the direct antiglobulin test (DAT); however, IgM and particularly IgA antibodies may not necessarily be detected by the broad-spectrum anti-human-globulin serum. We present the case of a 48-yr-old woman with severe AIHA. The initial polyspecific direct antiglobulin test (DAT), using a broad-spectrum antiserum, was negative. Testing with monospecific antisera led to the diagnosis of AIHA due to warm-acting auto-antibodies solely of the IgA class, which is a very rare finding. As therapy with steroids alone did not lead to a lasting remission, splenectomy was performed 10 months after initial diagnosis. There has been no relapse of AIHA since, even after steroid medication was withdrawn and even though the monospecific IgA-DAT has remained positive. This case demonstrates the importance of performing a monospecific antiglobulin test if there is a strong suspicion of AIHA in apparently "Coombs-negative" haemolytic anaemia. In AIHA caused by solely IgA antibodies, the polyspecific direct antiglobulin test may be negative or only weakly positive because of a limited content of anti-IgA antibodies in the polyspecific anti-human-globulin serum. First-line treatment of warm-type AIHA is the administration of high-dose glucocorticosteroids; splenectomy is indicated in steroid-refractory patients.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Bardill B,Mengis C,Tschopp M,Wuillemin WAdoi
10.1034/j.1600-0609.2003.02846.xsubject
Has Abstractpub_date
2003-01-01 00:00:00pages
60-3issue
1eissn
0902-4441issn
1600-0609pii
2c864journal_volume
70pub_type
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journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1034/j.1600-0609.2002.01635.x
更新日期:2002-05-01 00:00:00
abstract::Many cost analyses of stem-cell transplantations are available, which is in sharp contrast to the level of cost analyses on first-line chemotherapy for aggressive non-Hodgkin's lymphoma (NHL). Given the scarcity of cost analyses of first-line chemotherapy for NHL, it is difficult to assess the economic impact of upcom...
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journal_title:European journal of haematology
pub_type: 杂志文章
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journal_title:European journal of haematology
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journal_title:European journal of haematology
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更新日期:2015-02-16 00:00:00
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pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00362.x
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pub_type: 杂志文章
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更新日期:2016-07-01 00:00:00
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更新日期:2011-01-01 00:00:00
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journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
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更新日期:1991-11-01 00:00:00
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pub_type: 杂志文章
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更新日期:2014-06-01 00:00:00
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pub_type: 杂志文章
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更新日期:2003-02-01 00:00:00
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journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
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