European journal of haematology
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Vaso-occlusive crisis (VOC) is a significant cause of morbidity and mortality in sickle cell anemia (SCA) patients. Insofar as polymorphism in human platelet alloantigen (HPA) exhibit a prothrombotic nature, we hypothesized that specific HPA polymorphic variants are associated with VOC. We investigated the distribution of HPA1, HPA2, HPA3, HPA4, and HPA5 alleles genotypes among VOC and non-VOC control SCA patients. ⋯ This confirms the association of HPA polymorphisms with SCA VOC, of which HPA-3 appears to be independent genetic risk factors for SCA VOC.
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Nursing in 'live islands' and routine high dose intravenous immunoglobulins after allogeneic hematopoietic stem cell transplantation were abandoned by many teams in view of limited evidence and high costs. ⋯ Change from nursing in 'live islands' to SR and reduction of high dose to targeted IVIG did not result in increased infection rates or mortality despite an increase in patient age. These results support the current practice.
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Review Comparative Study
Laboratory diagnosis of the antiphospholipid syndrome: a plethora of obstacles to overcome.
The antiphospholipid antibody syndrome (APS) is defined by two major elements: the presence in plasma of auto-antibodies, i.e. antiphospholipid antibodies and the occurrence of clinical features, categorised as vascular thrombosis or pregnancy morbidity. In contrast to recent reviews on the physiopathology of APS, the present review focuses on the laboratory diagnosis of APS. ⋯ The highlight in this review will therefore be on the potential and limitations of the detection of the lupus anticoagulant, as an established laboratory criterion for the diagnosis of APS. The strengths and weaknesses of the current laboratory guidelines are discussed against our current insight in the physiopathology of APS.
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Enhanced bronchoalveolar coagulation is a hallmark of many acute inflammatory lung diseases such as acute lung injury, acute respiratory distress syndrome and pneumonia. Intervention with natural anticoagulants in these diseases has therefore become a topic of interest. ⋯ The aim of this review is to summarize these findings. Furthermore, the results of anticoagulant therapeutic interventions in these disorders are discussed.
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Contact factor pathway deficiencies do not cause surgical bleeding but make heparin monitoring by the activated partial thromboplastin time (APTT) and activated clotting time (ACT) unreliable. Heparin monitoring during cardiopulmonary bypass (CPB) surgery in these patients is particularly challenging. Here we describe heparin monitoring during CPB using the chromogenic anti Xa assay in two patients with severe factor XII deficiency (FXII < 0.01 U/mL) and one patient with severe prekallikrein (PK) deficiency (PK < 0.01 U/mL). ⋯ We conclude that the sensitivity of APTT methods for contact pathway deficiencies is highly variable and although insensitivity is not a clinical problem in terms of bleeding, it can be a cause of discrepancy between different APTT reagents and the ACT. This can lead to confusion about a possible haemorrhagic tendency and delays in surgery. If these patients need to undergo cardiac surgery requiring high dose heparin treatment, monitoring by chromogenic anti Xa assay is a good alternative.