Disseminated intravascular coagulation D65.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.03.2021

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Synonym(s)

Consumption coagulopathy; Defibrination syndrome; DIC; Disseminated coagulopathy; Disseminated intravascular coagulation; Disseminated intravascular coagulopathy; Disseminated vasal coagulation; Vasal coagulation disseminated

History
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Dupuy, 1834

Definition
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Acquired breakdown of hemostasis involving platelets, epithelium, coagulation factors, fibrinolysis, inhibitors, complement and the kininogen system. The result is acute intravascular coagulation of varying aetiology with formation of numerous microthrombi, consumption of all coagulation active substances (platelets, fibrinogen, coagulation factors) and a consecutive tendency to bleed.

Etiopathogenesis
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Disseminated intravascular coagulation (DIC) is a disorder that can have many causes, all of which converge on a common etiopathogenetic final pathway. The basis of the disorder is a consumption of platelets, coagulants, and anticoagulants to an extent that can no longer be compensated by the synthesis of these substances . Therefore, localized or generalized hemorrhagic diatheses, thromboses in the microcirculation, tissue necrosis and multiple organ failures occur.

Triggers are often bacterial infections by streptococci, staphylococci, pneumococci, meningococci, gram-negative rods, but also viruses, parasites, rickettsiae or mycoses. Other causes are endothelial damage, e.g. in the context of a haemolytic-uraemic syndrome, acute glomerulonephritis or Rocky Mountain fever.

Other feared causes are obstetric complications such as placental abruption, amniotic fluid embolism, septic abortion, intrauterine amniotic death and eclampsia.

Other frequent causes are neoplasms such as adenocarcinomas, acute promyelocytic leukaemia, but also severe haemolyses. Furthermore, tissue damage, such as burns, frostbite and gunshot wounds, can trigger a consumption coagulopathy.

An important cause of DIC is acute promyelocytic leukemia. The leukemic promyelocytes produce a leukocyte elastase that proteolytically destroys alpha-2 antiplasmin, factor-V, fibrin, and plasmin. In addition, promyelocytes produce t-PA and urokinase.

Several fatal cases of disseminated intravascular coagulation have occurred during the current COVID-19 vaccination campaigns with Astra-Seneca 's vector vaccine . The fatal disseminated coagulation episodes occurred within a 14-day period after vaccination. Persons <55 years of age were affected more frequently. Women were affected more frequently ( EMA communication of 18.3.2021).

Clinical features
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Skin lesions (in 70% of patients): Variable picture from acral cyanosis to small spotted purpura to extensive bleeding with hemorrhagic blisters and necroses.

Laboratory
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SPA increase, anaemia, thrombocyte lowering, Quick, fibrinogen and coagulation factors (factors V, VIII, XI).

Therapy
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Treatment of the underlying disease, heparinization after thrombin time, substitution of coagulation factors. In case of heparin allergy, see also Lepirudin, see also Hirudin.

Note(s)
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A cause of DIC that is rare in Europe is the venom of the Malayan moccasin viper. It contains enzymes that destroy fibrin and have thrombin-like effects. These effects are not antagonizable by AT-III.

Literature
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  1. Chang JC (2018) Disseminated intravascular coagulation: is it fact or fancy?
    Blood Coagul Fibrinolysis 29:330-337.
  2. Dupuy M (1834) Injections de matière cérébrale dans les veines. Gaz Med Paris 2: 524
  3. Hengge UR et al (2002) Purpura fulminans. A fatal consequence of a widely used medication? dermatologist 53: 483-487
  4. Levi M et al (2003) Sepsis and disseminated intravascular coagulation. J Thromb thrombolysis 16: 43-47
  5. Lubach D, Barthels M (1984) Two-dimensional purpura with necroses in severe cases of consumption coagulopathy. Dermatologist 35: 152-158
  6. Rheingold SR et al (2004) HIV infection presenting as severe autoimmune hemolytic anemia with disseminated intravascular coagulation in an infant. J Pediatr Hematol Oncol 26: 9-12
  7. Slofstra SH et al (2003) Disseminated intravascular coagulation. Hematol J 4: 295-302
  8. Toh CH et al (2003) Disseminated intravascular coagulation: old disease, new hope. BMJ 327: 974-977
  9. Veiga AB et al (2003) Fibrinogenolytic and procoagulant activities in the hemorrhagic syndrome caused by Lonomia obliqua caterpillars. Thromb Res 111: 95-101

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Last updated on: 18.03.2021