Best practice & research. Clinical haematology
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Indications for fresh frozen plasma (FFP), once used routinely in the support of critically ill infants and children, have become more specific as evolving evidence has confirmed or disproved the efficacy of plasma in various circumstances. FFP is currently indicated to treat the coagulopathies of massive hemorrhage, liver failure and disseminated intravascular coagulation and sepsis. Whole blood reconstituted from FFP and packed red cells is the product of choice for exchange transfusion, as well as for circuit priming. ⋯ Cryoprecipitate is used chiefly as a source of fibrinogen, factor VIII and factor XIII in consumptive coagulopathy; recombinant or viral inactivated plasma derivatives are preferred for congenital deficiencies of factor VIII and von Willebrand factor. Recombinant and highly purified, viral inactivated, plasma-derived proteins are preferred over FFP for congenital and acquired deficiencies. This chapter reviews evidence to support the use of plasma and plasma derivatives for pediatric patients.
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Best Pract Res Clin Haematol · Jan 2006
ReviewPlasma and plasma components in the management of disseminated intravascular coagulation.
A variety of clinical conditions can cause systemic activation of coagulation that ranges from insignificant laboratory changes to severe disseminated intravascular coagulation (DIC). DIC consists of a widespread systemic activation of coagulation, resulting in diffuse fibrin deposition in small and midsize vessels. There is compelling evidence from clinical and experimental studies that DIC is involved in the pathogenesis of microvascular dysfunction and contributes to organ failure. ⋯ Recent insight into important pathogenetic mechanisms that might lead to DIC has resulted in novel preventive and therapeutic approaches to patients with sepsis and derangement of coagulation. Supportive strategies aimed at inhibition of coagulation activation might theoretically be justified and have been found beneficial in experimental and initial clinical studies. These strategies comprise inhibition of tissue factor-mediated activation of coagulation or restoration of physiological anticoagulant pathways.
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Best Pract Res Clin Haematol · Jan 2006
ReviewPlasma and plasma products in the treatment of massive haemorrhage.
Massive haemorrhage requires the use of plasma products when it is accompanied by a coagulopathy or when the more than one blood volume has been lost and intractable bleeding continues. The coagulopathy results from haemorrhagic shock, hypothermia, and activation, consumption and dilution of coagulation factors. Plasma products have a critical role in maintaining sufficient levels of coagulation proteins to ensure haemostasis can occur. ⋯ Recombinant factor VIIa has now been shown to have a role in massive haemorrhage. Randomised controlled trials are currently underway to determine the optimal dose and timing of its administration. The physiology and management of the coagulation disturbance using plasma products in the massive haemorrhage of specific clinical situations are described.
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Multiple myeloma is a treatable but not necessarily a curable plasma-cell cancer. After decades of minimal progress, two new classes of drugs with novel mechanisms of action - immunomodulatory drugs (thalidomide and lenalidomide) and proteasome inhibitors (bortezomib) - have been introduced for the treatment of this disease. Thalidomide and lenalidomide have shown great activity as single agents and in combination with glucocorticoids for the treatment of chemotherapy-refractory myeloma. ⋯ These drugs can easily be combined with other chemotherapeutic agents to potentiate the anti-myeloma effect. The immunomodulatory function of these drugs can be successfully exploited to control residual disease during remission. Thus, both thalidomide and lenalidomide have ushered in a new era of optimism in the management of this incurable cancer.