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- Ljung, Rolf
(författare)
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Prophylactic therapy in haemophilia.
- 2009
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Ingår i: Blood Reviews. - : Elsevier BV. - 1532-1681 .- 0268-960X. ; 23:6, s. 267-274
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Forskningsöversikt (refereegranskat)abstract
- Clinical experience since decades and numerous retro- and, recently, also prospective studies clearly demonstrate that prophylactic treatment, albeit much more expensive, is superior to on-demand treatment regardless if outcome focus on number of joint- or life-threatening bleeds or arthropathy, evaluated by X-ray or MRI, or quality of life measured by general or hemophilia specific instruments. Optimal prophylactic treatment should be started early (primary prophylaxis) but various opinions exist on the dose and dose interval, depending on the objective of treatment in the individual patient which in turn is usually dependent on the resources in the health care system. Secondary prophylaxis, started later in childhood or in adults is beneficial but less cost-effective. This review covers proof of concept of primary prophylaxis in children and secondary prophylaxis in adults, comparisons between prophylaxis and on demand treatment as well as outcome measurers, health economics and future trends of prophylactic treatment of hemophilia.
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- Qadri, Syed M., et al.
(författare)
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Eryptosis in health and disease : A paradigm shift towards understanding the (patho)physiological implications of programmed cell death of erythrocytes
- 2017
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Ingår i: Blood reviews. - : CHURCHILL LIVINGSTONE. - 0268-960X .- 1532-1681. ; 31:6, s. 349-361
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Forskningsöversikt (refereegranskat)abstract
- During the course of their natural ageing and upon injury, anucleate erythrocytes can undergo an unconventional apoptosis-like cell death, termed eryptosis. Eryptotic erythrocytes display a plethora of morphological alterations including volume reduction, membrane blebbing and breakdown of the membrane phospholipid asymmetry resulting in phosphatidylserine externalization which, in turn, mediates their phagocytic recognition and clearance from the circulation. Overall, the eryptosis machinery is tightly orchestrated by a wide array of endogenous mediators, ion channels, membrane receptors, and a host of intracellular signaling proteins. Enhanced eryptosis shortens the lifespan of circulating erythrocytes and confers a procoagulant phenotype; this phenomenon has been tangibly implicated in the pathogenesis of anemia, deranged microcirculation, and increased prothrombotic risk associated with a multitude of clinical conditions. Herein, we reviewed the molecular mechanisms dictating eryptosis and erythrophagocytosis and critically analyzed the current evidence leading to the pathophysiological ramifications of eryptotic cell death in the context of human disease.
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- Saghafian-Hedengren, Shanie, et al.
(författare)
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Insights into defective serological memory after acute lymphoblastic leukaemia treatment : The role of the plasma cell survival niche, memory B-cells and gut microbiota in vaccine responses
- 2018
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Ingår i: Blood reviews. - : Elsevier BV. - 0268-960X .- 1532-1681. ; 32:1, s. 71-80
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Forskningsöversikt (refereegranskat)abstract
- Acute lymphoblastic leukaemia (ALL) is the most common type of cancer in children, accounting for approximately 25% of childhood cancer cases. As a result of effective treatments over the past decades, paediatric ALL mortality has been greatly reduced. Chemotherapy, however, has a range of harmful side effects including the loss of protective antibodies against vaccine-preventable diseases. Since ALL survivors have an increased risk of health problems including organ insufficiencies, acquired vaccine-preventable infections subsequent to clinical remission could become life threatening to these individuals. This review will summarize clinical findings regarding defective humoral immunity in ALL survivors, identify current knowledge gaps and highlight mechanisms related to deficiencies in the B-cell compartment important for serological memory. Further, we illuminate the emerging evidence for a relationship between chemotherapy and gut microbiota, which could play an important role in vaccine responses and the shaping of a young immune system subjected to maturation and recovery.
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- Svensson, Peter, et al.
(författare)
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Desmopressin in treatment of haematological disorders and in prevention of surgical bleeding.
- 2014
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Ingår i: Blood Reviews. - : Elsevier BV. - 1532-1681 .- 0268-960X. ; 28:3, s. 95-102
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Forskningsöversikt (refereegranskat)abstract
- Stimulation with the vasopressin analogue desmopressin (DDAVP) of extrarenal arginine vasopressin (AVP) V2-receptors in endothelial cells and possible in platelets increases the circulating levels of coagulation factor VIII (FVIII), von Willebrand factor (VWF) and tissue plasminogen activator (t-PA). The purpose of this paper is to provide an updated review of current information on the efficacy and safety of DDAVP in the treatment of haemophilia, von Willebrand disease (VWD), uremia, liver cirrhosis, and in congenital or drug-induced platelet dysfunction - under surgical or non-surgical conditions. In summary, desmopressin is an effective haemostatic drug that when administered i.v., s.c. or intranasally increases plasma levels of FVIII and VWF 2-6 times and improves platelet function. It has a proven haemostatic efficacy in mild haemophilia A and VWD as well as in uremia, liver cirrhosis and in congenital and acquired, drug induced platelet dysfunction. Desmopressin has few side effects but observation is advised in small children and elderly.
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