Treatment of the underlying disorders
Active treatment of the underlying disorder is key as this stops the triggering process.[10]Levi M, Toh CH, Thachil J, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009 Apr;145(1):24-33.
https://onlinelibrary.wiley.com/doi/10.1111/j.1365-2141.2009.07600.x
http://www.ncbi.nlm.nih.gov/pubmed/19222477?tool=bestpractice.com
In some cases, such as abruptio placentae, disseminated intravascular coagulation (DIC) will quickly resolve itself after elimination of the underlying condition. However, in other cases, such as sepsis, DIC may become more severe, even after effective treatment has been instituted. In those cases, other supportive measures, including replacement therapy and restoration of normal coagulant pathways, should be considered.[1]Levi M, De Jonge E, van der Poll T. New treatment strategies for disseminated intravascular coagulation based on current understanding of the pathophysiology. Ann Med. 2004;36(1):41-9.
http://www.ncbi.nlm.nih.gov/pubmed/15000346?tool=bestpractice.com
[2]Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006 Feb 21;4:4.
http://www.thrombosisjournal.com/content/4/1/4
http://www.ncbi.nlm.nih.gov/pubmed/16504043?tool=bestpractice.com
Restoration of physiologic coagulation pathways
Normal coagulation can be restored by inhibition of overactive coagulation pathways and enhancement of suppressed anticoagulation systems.
Heparin can be considered for this purpose; it inhibits the coagulation cascade and prevents further thrombogenesis by enhancing the effect of antithrombin III. Thus, normal levels of antithrombin III are a prerequisite for heparin to be effective.[16]Lemmer JH Jr, Despotis GJ. Antithrombin III concentrate to treat heparin resistance in patients undergoing cardiac surgery. J Thorac Cardiovasc Surg. 2002 Feb;123(2):213-7.
https://www.jtcvs.org/article/S0022-5223(02)77890-2/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/11828278?tool=bestpractice.com
Heparin may be indicated in selected patients with dominant symptoms and signs of thrombosis without evidence of significant bleeding, especially in the case of chronic DIC.[2]Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006 Feb 21;4:4.
http://www.thrombosisjournal.com/content/4/1/4
http://www.ncbi.nlm.nih.gov/pubmed/16504043?tool=bestpractice.com
[7]Levi M, Scully M. How I treat disseminated intravascular coagulation. Blood. 2018 Feb 22;131(8):845-54.
https://ashpublications.org/blood/article-lookup/doi/10.1182/blood-2017-10-804096
http://www.ncbi.nlm.nih.gov/pubmed/29255070?tool=bestpractice.com
[10]Levi M, Toh CH, Thachil J, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009 Apr;145(1):24-33.
https://onlinelibrary.wiley.com/doi/10.1111/j.1365-2141.2009.07600.x
http://www.ncbi.nlm.nih.gov/pubmed/19222477?tool=bestpractice.com
Heparin use is not advocated in patients at high risk of bleeding because it may worsen the bleeding problems associated with DIC.[7]Levi M, Scully M. How I treat disseminated intravascular coagulation. Blood. 2018 Feb 22;131(8):845-54.
https://ashpublications.org/blood/article-lookup/doi/10.1182/blood-2017-10-804096
http://www.ncbi.nlm.nih.gov/pubmed/29255070?tool=bestpractice.com
Replacement therapy
The aim of replacement therapy in DIC is to replace the deficient platelets and coagulation factors. Replacement of platelets and coagulation factors is indicated only in patients:
With active bleeding
Requiring an invasive procedure
At risk for bleeding complications
With a documented deficiency of platelets and/or coagulation factors/inhibitors.[2]Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006 Feb 21;4:4.
http://www.thrombosisjournal.com/content/4/1/4
http://www.ncbi.nlm.nih.gov/pubmed/16504043?tool=bestpractice.com
A patient with DIC is considered at high risk of bleeding if: a general risk for bleeding is found; the patient is about to undergo an invasive procedure; or there is documented deficiency of platelets and/or coagulation factors.
Risk assessment for DIC may be used as a surrogate measure of bleeding risk. Global coagulation tests should be ordered: platelet count; D-dimer/fibrin degradation products; prothrombin time; and fibrinogen. A specific score, reflecting the severity of abnormality, is given to each of the tests.
Fresh frozen plasma (FFP) and platelet concentrates are the two common blood components used to replace the deficient platelets and coagulation factors. FFP can provide coagulation factors and fibrinogen, as well as coagulation inhibitors in balanced amounts. Other blood products, such as cryoprecipitates and fibrinogen concentrates, may be considered, but FFP is preferable in the setting of DIC because it provides balanced coagulation factors and inhibitors.[1]Levi M, De Jonge E, van der Poll T. New treatment strategies for disseminated intravascular coagulation based on current understanding of the pathophysiology. Ann Med. 2004;36(1):41-9.
http://www.ncbi.nlm.nih.gov/pubmed/15000346?tool=bestpractice.com
[2]Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006 Feb 21;4:4.
http://www.thrombosisjournal.com/content/4/1/4
http://www.ncbi.nlm.nih.gov/pubmed/16504043?tool=bestpractice.com
Antifibrinolytic agents
Antifibrinolytic agents, such as aminocaproic acid and tranexamic acid, should be used with extreme caution because they inhibit the fibrinolytic pathways, which may worsen the symptoms and signs of thrombosis. They are occasionally used in patients with severe refractory bleeding resistant to conventional replacement therapy or in patients with hyperfibrinolysis associated with acute promyelocytic leukemia and other forms of cancer.[1]Levi M, De Jonge E, van der Poll T. New treatment strategies for disseminated intravascular coagulation based on current understanding of the pathophysiology. Ann Med. 2004;36(1):41-9.
http://www.ncbi.nlm.nih.gov/pubmed/15000346?tool=bestpractice.com
[2]Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006 Feb 21;4:4.
http://www.thrombosisjournal.com/content/4/1/4
http://www.ncbi.nlm.nih.gov/pubmed/16504043?tool=bestpractice.com
[10]Levi M, Toh CH, Thachil J, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009 Apr;145(1):24-33.
https://onlinelibrary.wiley.com/doi/10.1111/j.1365-2141.2009.07600.x
http://www.ncbi.nlm.nih.gov/pubmed/19222477?tool=bestpractice.com
[17]Avvisati G, ten Cate JW, Buller HR, et al. Tranexamic acid for control of hemorrhage in acute promyelocytic leukemia. Lancet. 1989 Feb 15;2(8455):122-4.
http://www.ncbi.nlm.nih.gov/pubmed/2567893?tool=bestpractice.com