Best practice & research. Clinical haematology
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Best Pract Res Clin Haematol · Sep 2012
ReviewAdvances in the diagnosis and management of postthrombotic syndrome.
Postthrombotic syndrome (PTS) is a frequent long-term complication of deep vein thrombosis (DVT). Known risk factors include obesity, recurrent ipsilateral DVT, iliofemoral DVT, persistent symptoms one month after DVT diagnosis, and having subtherapeutic INRs greater than 50% of the time during the first few months on anticoagulant therapy. Other risk factors remain under investigation. ⋯ Though not well studied, there are surgical options for severe cases of PTS refractory to other treatments. Ongoing clinical trials should provide insight on risk factors, and interventions for PTS prevention and treatment. In particular, use of early thrombolysis for acute iliofemoral DVT to prevent PTS is currently being investigated.
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Best Pract Res Clin Haematol · Jun 2010
ReviewDouble cord blood transplantation: extending the use of unrelated umbilical cord blood cells for patients with hematological diseases.
Unrelated umbilical cord blood (UCB) has been widely used to treat patients lacking a well-matched HLA donor. Cell dose is a critical determinant of outcomes in cord blood transplantation, limiting the use of this strategy for low body weight patients. ⋯ Approximately 993 adults with hematological diseases have been transplanted with double UCB graft, and reported to Eurocord registry from 1999 to 2010. This article reviews the state of art and future directions with double umbilical cord blood units as a source of hematopoietic stem cells for transplantation.
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Best Pract Res Clin Haematol · Dec 2009
ReviewThrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies.
Thrombotoic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterised by systemic platelet aggregation, organ ischaemia, profound thrombocytopenia (with increased marrow megakaryocytes) and fragmentation of erythrocytes. Haemolytic-uraemic syndrome (HUS) is another type of thrombotic microangiopathy accompanied by renal dysfunction. ⋯ This article discusses the thrombotic microangiopathies that have provided the most extensive molecular insights to date into pathophysiology. These are familial and acquired forms of TTP associated with deficient plasma von Willebrand factor (VWF)-cleaving metalloprotease (ADAMTS-13) activity; acquired diarrhoea-associated HUS; the thrombotic microangiopathies associated with cyclosporine/tacrolimus or bevacizumab; and the preeclampsia-HELLP (haemolysis-elevated liver enzymes-low platelets) syndrome.
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Best Pract Res Clin Haematol · Jun 2008
ReviewAcute graft-versus-host disease: differing risk with differing graft sources and conditioning intensity.
Acute graft-versus-host disease (aGVHD) is a constant component of allogeneic hematopoietic cell transplantation (HCT), with variations in incidence and severity affected by the graft source, human leukocyte antigen (HLA) compatibility, and the preparative regimen. The graft source - related versus unrelated donors, bone marrow (BM) versus peripheral blood (PB), umbilical cord blood (UCB) versus unrelated donor BM - are discussed in this review, as well as myeloablative versus reduced-intensity (RI) preparative regimens. Recent comparisons of matched related versus matched unrelated donor HCT support a minimal difference in aGVHD between these two donor sources. ⋯ Haploidentical HCT has continued to be explored, with limitations due to delayed immune reconstitution and disease relapse. Many reduced-intensity preparative regimens have been published, with a reduced or minimal difference in incidence of aGVHD when historically compared to myleoablative preparative regimens. More formal comparisons of the different graft sources as well as preparative regimen intensities will be required to determine a more accurate picture of the differences between these transplantation alternatives.