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Paeds Caseshaematology-oncology-and-transfusion

Paeds Cases · haematology-oncology-and-transfusion

Disseminated intravascular coagulation: Case

Clinical case of a child with sepsis-induced disseminated intravascular coagulation presenting with purpura fulminans, covering the ISTH overt-disseminated intravascular coagulation score of five points or more, the cause-driven management with the broad-spectrum antibiotics and the source control, the blood components reserved for the bleeding child, and the heparin for the thrombosis-dominated course.

paediatric haematology critical-care case4 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A previously well three-year-old girl is brought to the emergency department with a twelve-hour history of fever, irritability, and a rapidly evolving rash. She is tachycardic at 160 per minute, hypotensive at 70 over 40 millimetres of mercury, and peripherally shut down, with cool dusky digits and large stellate purpuric lesions over the limbs and the trunk that are turning necrotic. She is confused. The full blood count shows a haemoglobin of 96 grams per litre, a white cell count of 22 times ten to the nine per litre, and a platelet count of 34 times ten to the nine per litre. The prothrombin time is prolonged at 22 seconds, the fibrinogen is 0.7 grams per litre, and the D-dimer is strongly raised. The blood film shows fragmented red cells. There is oozing from the cannula site.

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]

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References3ShowHide
  1. [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. [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. [3]Rajagopal R, Thachil J, Monagle P Disseminated intravascular coagulation in paediatrics Arch Dis Child, 2017.PMID 27540263
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