Primary mediastinal B cell lymphoma (PMBCL) is a rare type of non-Hodgkin's lymphoma, and prompt diagnosis and initiation of chemotherapy are necessary to limit compression of cardiovascular structures. A 13-year-old patient was diagnosed with PMBCL, resulting in acute hypoxemic respiratory failure and cardiogenic shock with clinical pericardial tamponade. Chemotherapy was initiated after veno-arterial extracorporeal membrane oxygenation and subsequent endovascular stenting of the superior vena cava (SVC) to optimize cardiac output due to malignant SVC syndrome. We discuss the challenges of diagnosis and emergency management of pediatric patients with PMBCL and review the existing relevant literature for SVC syndrome secondary to PMBCL.
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Maffei et al. (2024) studied this question.
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