Abstract:BackgroundTMA is a life threatening complication with a high mortality rate in SCT patients.1 The pathophysiology involves damage of the endothelial lining via over activation of the complement system which forms part of the innate immune system.2 Complications include; kidney damage, pulmonary hypertension and possible multi organ failure.1 Eculizumab is an agent that exhibits activity by inhibiting the complement system however dosing and effects in the paediatric population are lacking.1,2 An 18 year old pa… Show more
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