Disseminated intravascular coagulation: SAQ

This boy has the acute, fulminant, thrombosis-dominated disseminated intravascular coagulation of meningococcal sepsis. He 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 ISTH overt-DIC score is well above five points, and the management rests on the cause-driven principle. ^[1][2][3]

Question 1 (10 marks)

Outline the diagnosis, the immediate management, and the resuscitation for this boy, justifying each step with the relevant evidence. ^[2]

A full-mark answer addresses the ISTH score, the cause-driven resuscitation, the components for the bleeding, and the heparin for the purpura fulminans. ^[1]

Diagnosis and the ISTH overt-DIC score (3 marks). This is disseminated intravascular coagulation, an acquired, trigger-driven syndrome of the systemic intravascular coagulation. The ISTH overt-DIC score totals the platelet count, the fibrinogen, the D-dimer, and the prothrombin time, and a score of five points or more, in a child with a compatible trigger, defines the overt disease. This boy scores two for the platelets under fifty, one for the fibrinogen under one gram per litre, three for the strongly raised D-dimer, and at least one for the prolonged prothrombin time, for a total well above five, and the septic shock with the purpura fulminans is the trigger. The fragmented red cells on the film are the microangiopathic haemolysis. ^[1][2]

The cause-driven resuscitation (4 marks). The definitive treatment of disseminated intravascular coagulation is the treatment of the trigger, and this boy 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 DIC does not resolve until the trigger is removed, and every other measure buys time for the cause treatment to work. The British Committee for Standards in Haematology guideline of Levi and colleagues sets out this cause-driven principle, and the fellow who treats the cause first holds the central management principle. ^[2]

The components and the heparin (3 marks). The blood components treat the bleeding, and the boy 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. Because the picture is thrombosis-dominated, with the purpura fulminans and the digital ischaemia, the boy also receives the therapeutic unfractionated heparin, titrated to the partial thromboplastin time, alongside the urgent plastic-surgical review for the skin and the limb salvage. The components and the heparin support the child while the cause treatment resolves the DIC. ^[2][3]