Paeds Vivas · haematology-oncology-and-transfusion
Disseminated intravascular coagulation: Viva
Branching clinical structured oral on disseminated intravascular coagulation in children, covering the trigger-driven acquired syndrome, the ISTH overt-disseminated intravascular coagulation score of five points or more, the bleeding-and-thrombosis paradox, the cause-driven management with the blood components reserved for the bleeding child, and the heparin for the thrombosis-dominated purpura fulminans, appraising the sepsis-induced coagulopathy score and the anticoagulant evidence.
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This is the acute, fulminant, thrombosis-dominated disseminated intravascular coagulation of meningococcal sepsis. The structured oral runs from the ISTH score through the cause-driven resuscitation, the components for the bleeding, the heparin for the purpura fulminans, and the anticoagulant evidence. [1][2]
Opening: make the diagnosis
Examiner. The platelet count is 38 times ten to the nine per litre, the fibrinogen is 0.8 grams per litre, and the D-dimer is strongly raised. What is the diagnosis, and how do you confirm it? [1]
Candidate. This is disseminated intravascular coagulation, an acquired, trigger-driven syndrome of the systemic intravascular coagulation, and the trigger here is the septic shock with the purpura fulminans. I confirm it with the ISTH overt-DIC score, which totals the platelet count, the fibrinogen, the D-dimer, and the prothrombin time. 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, the threshold for the overt disease. The fragmented red cells on the film are the microangiopathic haemolysis. [1][2]
Examiner (probe). What would make you look for a different trigger? [3]
Candidate. The triggers in children are the sepsis, the malignancy, the trauma, and the obstetric and neonatal catastrophes. If the child had the bruising and the pallor with the blast cells on the film, I would suspect the acute leukaemia, especially the acute promyelocytic leukaemia. If the child had the major trauma or the burns, I would recognise the tissue-factor release. The chronic, low-grade picture with the isolated thrombocytopenia would raise the Kasabach-Merritt haemangioma. The trigger drives both the cause treatment and the prognosis. [3]
References4ShowHide
- [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]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
- [3]Rajagopal R, Thachil J, Monagle P Disseminated intravascular coagulation in paediatrics Arch Dis Child, 2017.PMID 27540263
- [4]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