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Acquired haemophilia Aerrors in the diagnosis

 

作者: M. Kazmi,   W. Pickering,   M. Smith,   L. Holland,   G. Savidge,  

 

期刊: Blood Coagulation and Fibrinolysis  (OVID Available online 1998)
卷期: Volume 9, issue 7  

页码: 623-628

 

ISSN:0957-5235

 

年代: 1998

 

出版商: OVID

 

关键词: inhibitor;antiphospholipid antibodies;acquired haemophilia;systemic lupus erythematosus

 

数据来源: OVID

 

摘要:

The distinction between a specific factor inactivator and a non-specific inhibitor is important when confronted by a patient with a history of bleeding and abnormal in-vitro coagulation tests. We report on two patients who presented with bleeding and a prolonged activated partial thromboplastin time. Initial factor assays suggested combined deficiency of factors VIII and IX as a result of the presence of inactivators. The use of dilution studies, chromogenic assays, a novel in-house enzyme-linked-immunosorbent-assay-based technique and phospholipid neutralization, demonstrated that Case 1 had a genuine factor VIII inactivator resulting in factor VIII levels of less than 1 IU/dl but no factor IX deficiency. Case 2 had normal levels of factor VIII on further testing and no specific inactivator to either factor VIII or IX but a potent antiphospholipid antibody which had interfered with the phospholipid-dependent in-vitro assays. Care must be taken in the interpretation of laboratory assays in the presence of antiphospholipid antibodies to ensure that the correct diagnosis is made and inappropriate treatment avoided.

 

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