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Kasabach-Merritt phenomenon — RACP Paediatrics MCQ

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HardNeonatalKasabach-Merritt phenomenonRACP Paediatrics

A neonate has thrombocytopenia (platelets 60) in the context of a large rapidly growing cutaneous vascular lesion (kaposiform haemangioendothelioma) on the trunk. There is also a consumptive coagulopathy with elevated D-dimer and low fibrinogen. What is the most likely cause?

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Correct answer: B — Kasabach-Merritt syndrome

Kasabach-Merritt phenomenon (consumptive coagulopathy with thrombocytopenia) is associated with kaposiform haemangioendothelioma or tufted angioma – NOT with common infantile haemangiomas. The tumour traps and consumes platelets and clotting factors. Treatment includes sirolimus, corticosteroids, and vincristine. Platelet transfusion is avoided unless active bleeding as it may fuel tumour growth.

Reference: RCH Melbourne – 2023 – Vascular Anomalies CPG; RACP Paediatric Curriculum – Neonatology