Disseminated intravascular coagulation: Case

This girl has the acute, fulminant, thrombosis-dominated disseminated intravascular coagulation of meningococcal sepsis. She is in septic shock, with the purpura fulminans and the digital ischaemia, the thrombocytopenia, the prolonged prothrombin time, the low fibrinogen, the strongly raised D-dimer, and the fragmented red cells on the film. The oozing cannula site is the bleeding face of the process, and the purpura fulminans is the thrombotic face. The ISTH overt-DIC score is well above five points. [1][2][3]

Interpretation of the findings

The key findings are the septic shock, the bleeding, and the thrombosis, set against the deranged coagulation screen. The platelet count of 34 times ten to the nine per litre is well under the threshold, and the prolonged prothrombin time, the low fibrinogen, and the strongly raised D-dimer together build the ISTH overt-DIC score to well above five points. The fragmented red cells on the film are the microangiopathic haemolysis, the hallmark of the microvascular fibrin shearing the red cells as they pass. The purpura fulminans and the cool dusky digits are the microvascular thrombosis that drives the organ failure. [1][2]

The fibrinogen of 0.7 grams per litre is low, and the fellow notes that even a normal fibrinogen in this septic child would not have been reassuring, because the fibrinogen is an acute-phase reactant and an inappropriately normal value may hide the consumption. The diagnosis of disseminated intravascular coagulation is therefore made on the combination of the septic shock, the bleeding, the thrombosis, the deranged screen, and the ISTH score of five points or more, and the management rests on the cause-driven principle. [2][3]

Management: treat the cause and resuscitate

The definitive treatment is the treatment of the trigger, and this girl has the septic shock, so the first action is the broad-spectrum antibiotics within the first hour, the fluid resuscitation, and the vasopressors, alongside the source control and the paediatric intensive-care referral. The British Committee for Standards in Haematology guideline of Levi and colleagues sets out this cause-driven principle, because the disseminated intravascular coagulation does not resolve until the trigger is removed, and every other measure buys time for the cause treatment to work. The girl is managed in the paediatric intensive-care unit throughout. [2]

The blood components treat the bleeding, and the girl receives the platelet transfusion for the count under fifty times ten to the nine per litre, the fresh-frozen plasma at ten to fifteen millilitres per kilogram for the prolonged clotting times, and the cryoprecipitate or the fibrinogen concentrate to hold the fibrinogen over one gram per litre. The oozing cannula site is the active bleeding that triggers the components. The components are not given prophylactically for the score, because the prophylactic transfusion does not change the outcome and it carries its own volume and transfusion risks. [2]

The thrombosis-dominated picture and the heparin

The purpura fulminans and the cool dusky digits shift the management toward the anticoagulation, because the thrombosis dominates the picture. The girl receives the therapeutic unfractionated heparin, titrated to the partial thromboplastin time, alongside the components and the cause treatment. The heparin is reserved for the thrombosis-dominated course, because the routine heparin for the bleeding-dominated disseminated intravascular coagulation is not supported by the evidence. The plastic surgery is involved early for the skin and the limb salvage, because the survivor may face the amputations and the skin grafting. [2][3]

The antithrombin concentrate is not given routinely, because the survival benefit has not been shown, and the recombinant activated protein C, the drotrecogin alfa, was withdrawn in 2011 after the PROWESS-SHOCK trial showed no benefit. The recombinant soluble thrombomodulin, the ART-123, is the newer agent, but the paediatric data are limited and it is not available in every region. The fellow holds the cause treatment, the components, and the heparin as the core of the management. [2]

The course and the counselling

The ISTH score is repeated daily, because the course of the disseminated intravascular coagulation is dynamic, and the score that falls signals the recovery while the score that climbs signals the worsening and the higher mortality. The girl is followed in the paediatric intensive-care unit, with the haematology and the infectious-diseases input, and the sepsis-induced coagulopathy score is added to track the upstream state that precedes the overt disease. [2][3]

The family is counselled on the high mortality, which sits around a quarter to a half in the severe, intensive-care cases and is driven by the underlying cause. The counselling is honest and it is compassionate, because the family faces a child in the intensive-care unit with a life-threatening illness and the prospect of the amputations and the skin grafting. The fellow explains the cause-driven management, the components, and the heparin, and the family is supported by the multidisciplinary team throughout the acute illness and the rehabilitation. The discharge is planned when the trigger is treated, the bleeding is controlled, the score has fallen, and the organ function has recovered. [2][3]