British journal of haematology
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We tested the hypothesis that extracellular haem is linked to the incidence of acute complications of sickle cell disease (SCD). Using multivariable regression analysis, higher plasma free haem, but not total plasma haem, was associated with increased odds of vaso-occlusive crisis (VOC) [P = 0·028, odds ratio (OR); 2·05, 95% Confidence Interval (CI) = 1·08-3·89] and acute chest syndrome (ACS) [P = 0·016, OR; 2·56, CI = 1·19, 5·47], after adjusting for age and gender in children with SCD. These findings suggest that haem and factors that influence its concentration in plasma may be informative of the risk of VOC and ACS in SCD patients.
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Although echocardiography-derived tricuspid regurgitant jet velocity (TRV) is associated with increased mortality in sickle cell disease (SCD), its rate of increase and predictive markers of its progression are unknown. We evaluated 55 subjects (median age: 38 years, range: 20-65 years) with at least two measurable TRVs, followed for a median of 4·5 years (range: 1·0-10·5 years) in a single-centre, prospective study. Thirty-one subjects (56%) showed an increase in TRV, while 24 subjects (44%) showed no change or a decrease in TRV. ⋯ In summary, although some patients have clinically meaningful increases, the overall rate of TRV increase is slow. Treatment with hydroxycarbamide may decrease the progression of TRV. Additional studies are required to determine the optimal frequency of screening echocardiography and the effect of therapeutic interventions on the progression of TRV in SCD.
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Central venous catheters are commonly used in haematology departments for the administration of chemotherapy, blood products and parenteral nutrition in patients with haematological malignancy. Thrombosis is a recognized complication of such devices especially in oncology patients. Catheter-related thrombi (CRT) may lead to pulmonary embolism and infection, as well as catheter failure and potential delays in treatment. ⋯ Doppler ultrasound or venography may be employed to identify CRT. Once confirmed, the initiation of treatment is a balance between the risks of anticoagulation against potential complications of CRT. A number of risk factors for CRT are discussed along with an overview of catheter types, their appropriate choice and intravascular positioning.