BMJ case reports · 2025
Fulminant fat embolism syndrome in paediatric sickle cell disease managed with extracorporeal membrane oxygenation and total plasma exchange.
СтатьяКлинический случайНазвание и аннотация: Перевод пока не подготовлен
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Cromb Daniel, Cousin Vladimir L, Pavcnik Maja, Boot Elizabeth, Fordham Nicholas, Babiker Samah
Аннотация
We present the case of a boy with sickle cell disease who developed fulminant fat embolism syndrome following parvovirus-B19 infection. After presenting with severe bone pain, his condition rapidly deteriorated, resulting in respiratory failure and multiorgan dysfunction. He was admitted to the paediatric intensive care unit for venovenous extracorporeal membrane oxygenation (VV-ECMO), in conjunction with red cell and plasma exchange transfusions. At a follow-up 12 months later, he had made a full neurological recovery.This case highlights the importance of early recognition of fat embolism syndrome in patients with sickle cell disease: the association of severe painful crises, neurological symptoms, elevated ferritin and thrombocytopenia, often preceded by parvovirus infection. It demonstrates the value of urgent, aggressive management and the need for multidisciplinary collaboration. This case is unique due to the paucity of cases reporting the use of ECMO in paediatric sickle cell disease and the limited paediatric data using plasma exchange in fat embolism syndrome.