Paeds · haematology-oncology-and-transfusion
Disseminated intravascular coagulation
Also known as DIC · Consumptive coagulopathy · Defibrination syndrome · Sepsis-induced coagulopathy · Purpura fulminans · Overt DIC
Fellowship guide to disseminated intravascular coagulation in children. Covers the acquired syndrome of systemic intravascular coagulation driven by sepsis, malignancy, trauma, and the obstetric causes, the ISTH overt-disseminated intravascular coagulation score that totals the platelet count, the fibrinogen, the D-dimer, and the prothrombin time and calls overt disease at five points or more, the bleeding-and-thrombosis paradox of microvascular fibrin with platelet and factor consumption, the management that treats the underlying cause first and adds platelet, plasma, and cryoprecipitate or fibrinogen concentrate for the bleeding child at platelets under fifty times ten to the nine per litre and fresh-frozen plasma at ten to fifteen millilitres per kilogram, the role of heparin in thrombosis-dominated purpura fulminans, and the withdrawal of drotrecogin alfa in 2011.
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- A child with sepsis or trauma who bleeds from venepuncture sites, mucosa, or lines, while also showing skin and digital ischaemia, has disseminated intravascular coagulation until the score and the cause are addressed
- Purpura fulminans, the large stellate purpuric lesions with skin and digital necrosis in meningococcal or pneumococcal sepsis, signals thrombosis-dominated disseminated intravascular coagulation and demands urgent plasma, heparin, and critical care
- A normal fibrinogen in a child with severe sepsis is not reassuring, because fibrinogen is an acute-phase reactant and an inappropriately normal value may hide consumption; a falling trend matters more than a single value
- Disseminated intravascular coagulation in acute promyelocytic leukaemia can precede the leukaemia diagnosis and is managed with all-trans retinoic acid started immediately, not delayed for the bone marrow result
- Do not give platelets, plasma, or cryoprecipitate prophylactically to a child who is not bleeding; treat the cause and reserve components for active bleeding or a high bleeding risk before procedures
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- Defines disseminated intravascular coagulation as an acquired syndrome of systemic intravascular coagulation arising from a trigger
- States the ISTH overt-disseminated intravascular coagulation score of five points or more
- Names sepsis, malignancy, trauma, and obstetric causes as the triggers
- Explains the bleeding-and-thrombosis paradox of microvascular fibrin with platelet and factor consumption
- Coordinates critical care, haematology, and the component and anticoagulant strategy
- Appraises the ISTH score, the sepsis-induced coagulopathy score, and the thrombomodulin and antithrombin evidence
- The ISTH overt-disseminated intravascular coagulation score totals platelets, fibrinogen, D-dimer, and prothrombin time, and five points or more defines overt disease
- Fresh-frozen plasma is ten to fifteen millilitres per kilogram, platelets are given for the bleeding child under fifty times ten to the nine per litre
- The central principle is to treat the underlying cause; components treat bleeding, not the score
- Builds the resuscitation around the cause, the bleeding, and the organ failure
- Counsels the family on the high mortality and the multisystem course
- Distinguishes the bleeding-dominated from the thrombosis-dominated picture and adjusts the plan
- Level 1: Recognition of the sick child with sepsis and the abnormal coagulation screen
- Level 2: The ISTH score, the cause-driven management, and the component thresholds
- Level 3: The purpura fulminans strategy and the multidisciplinary critical care
- The ISTH overt-disseminated intravascular coagulation score of five points or more
- The sepsis-induced coagulopathy score of four points or more as the precursor
- The component and anticoagulant doses and the causes including acute promyelocytic leukaemia
- Recognition of the bleeding-and-thrombosis paradox and the sick child
- Communication of the diagnosis, the severity, and the plan
- Recognition of purpura fulminans and the thrombosis-dominated course
- Disseminated intravascular coagulation as the consumptive coagulopathy of severe sepsis, malignancy, and trauma
- The ISTH score and the cause-driven management
- Acute promyelocytic leukaemia as the malignancy with the highest disseminated intravascular coagulation risk
- Recognition of the consumptive coagulopathy and the multisystem failure
- The ISTH score and the component and anticoagulant strategy
- The principle that treating the cause is the definitive treatment
- The sepsis-induced coagulopathy score and the thrombomodulin evidence
- The paediatric triggers including meningococcal sepsis and acute promyelocytic leukaemia
Overview & Definition
A previously well three-year-old presents with fever and a purpuric rash that has turned into large black stellate patches on the limbs, and the blood test shows a platelet count that has halved, a prothrombin time that is prolonged, and a fibrinogen that is lower than it should be for a child this sick. This is the face of disseminated intravascular coagulation in paediatrics, and it is one of the most feared emergencies in the intensive-care unit, because the same child bleeds from every line while the skin and the digits turn ischaemic. The fellow who understands that the bleeding and the thrombosis are two faces of the one process, and who reaches first for the cause rather than for the blood bank, is the fellow who manages this well. [4][5]
Disseminated intravascular coagulation, always abbreviated DIC, is an acquired syndrome in which the coagulation system is switched on systemically throughout the circulation, rather than locally at a wound. The Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis, in the consensus statement of Taylor and colleagues, defined it as an intravascular activation of coagulation that is no longer localised, and that arises from a clinical trigger and may cause organ damage. [1][2]
Three ideas make this topic central to the fellowship exam, and each is a deliberate principle rather than a reflex. The first is that DIC never exists alone, because it is always secondary to a trigger, and the triggers in children are sepsis, malignancy, major trauma, and the obstetric and neonatal catastrophes. The second is the scoring system, because the ISTH overt-DIC score of five points or more is the recognised way to turn a handful of abnormal blood tests into a working diagnosis. The third is the management, because the definitive treatment is to treat the cause, while the blood components and the anticoagulants are supportive measures that buy time. [3][5]
References12ShowHide
- [1]Taylor FB Jr, Toh CH, Hoots WK, Wada H Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost, 2001.PMID 11816725
- [2]Toh CH, Hoots WK The scoring system of the Scientific and Standardisation Committee on Disseminated Intravascular Coagulation of the International Society on Thrombosis and Haemostasis: a 5-year overview. J Thromb Haemost, 2007.PMID 17096704
- [3]Levi M, Toh CH, Thachil J, Watson HG Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol, 2009.PMID 19222477
- [4]Levi M, Ten Cate H Disseminated intravascular coagulation. N Engl J Med, 1999.PMID 10451465
- [5]Rajagopal R, Thachil J, Monagle P Disseminated intravascular coagulation in paediatrics. Arch Dis Child, 2017.PMID 27540263
- [6]Thachil J Disseminated intravascular coagulation - new pathophysiological concepts and impact on management. Expert Rev Hematol, 2016.PMID 27314681
- [7]Iba T, Nisio MD, Levy JH, et al. New criteria for sepsis-induced coagulopathy (SIC) following the revised sepsis definition: a retrospective analysis of a nationwide survey. BMJ Open, 2017.PMID 28963294
- [8]Iba T, Arakawa M, Di Nisio M, Gando S, Thachil J Newly proposed sepsis-induced coagulopathy precedes International Society on Thrombosis and Haemostasis overt-disseminated intravascular coagulation and predicts high mortality. J Intensive Care Med, 2020.PMID 29720054
- [9]Valeriani E, Squizzato A, Gallo A, Porreca E Efficacy and safety of recombinant human soluble thrombomodulin in patients with sepsis-associated coagulopathy: a systematic review and meta-analysis. J Thromb Haemost, 2020.PMID 32237269
- [10]Monagle P, Chalmers E, Chan A, DeVeber G, Kirkham F, Massicotte P, Michelson AD Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest, 2008.PMID 18574281
- [11]Umemura Y, Nishida T, Yamakawa K, Ogura H Anticoagulant therapies against sepsis-induced disseminated intravascular coagulation. Acute Med Surg, 2023.PMID 37670904
- [12]Ranieri VM, Thompson BT, Barie PS, et al. Drotrecogin alfa (activated) in adults with septic shock N Engl J Med, 2012.PMID 22616830