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HLH Etoposide — SCE Medical Oncology MCQ

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HardOncological EmergenciesHLH EtoposideSCE Medical Oncology

A patient with cancer develops fluctuating confusion, thrombocytopenia and microangiopathic haemolytic anaemia. Creatinine is only mildly raised, coagulation tests are normal and the pretreatment PLASMIC score is high. What is the most appropriate action while urgent ADAMTS13 testing is pending?

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Correct answer: D — Start plasma exchange, corticosteroids and caplacizumab with TTP-centre oversight

A high PLASMIC score with MAHA, thrombocytopenia and preserved coagulation warrants immediate plasma exchange, corticosteroids and caplacizumab under TTP-centre oversight while ADAMTS13 is pending. Start plasma exchange and corticosteroids while reserving caplacizumab for confirmed relapse: high-probability acute iTTP benefits from caplacizumab at presentation rather than after relapse. Start eculizumab and corticosteroids while urgent ADAMTS13 testing is processed: eculizumab is directed at complement-mediated aHUS, not high-probability iTTP. Start therapeutic plasma infusion and rituximab while urgent ADAMTS13 testing is processed: plasma infusion is inadequate compared with exchange in acquired iTTP. Start platelet transfusion and corticosteroids while urgent ADAMTS13 testing is processed: platelets can worsen microvascular thrombosis unless there is life-threatening bleeding.

Reference: British Society for Haematology guideline for TTP and thrombotic microangiopathies (Published August 2023; current BSH guidance): https://b-s-h.org.uk/guidelines/guidelines/diagnosis-and-management-of-thrombotic-thrombocytopenic-purpura-and-thrombotic-microangiopathies