Fostamatinib and the risk of acute aortic dissection in immune thrombocytopenia

Auteurs

Dalmas P, Theron A, Badaoui R, Zaffran S, Mchinda R, Micallef J, Schleinitz N, Ebbo M

Les auteurs en lien sont membres de l'INS.

Équipes

TNG

Résumé

Immune thrombocytopenia (ITP) is a rare autoimmune disorder characterized by platelet destruction. While most patients respond to first‐ or second‐line therapies, a small subset is multirefractory. Fostamatinib , an oral spleen tyrosine kinase ( SYK ) inhibitor, is a therapeutic option in these cases. We report a case of an acute aortic dissection (AAD) occurring in a patient without traditional cardiovascular risk factors, 3 months after fostamatinib initiation. A 70‐year‐old woman with a 30‐year history of ITP, unresponsive to multiple therapies, was treated with fostamatinib and achieved a complete haematologic response. She presented with sudden chest pain and was diagnosed with Stanford A (DeBakey type 1) AAD requiring emergency surgery. Histological analysis of her aortic tissue showed a 25% reduction in phosphorylated SYK expression compared to a control sample, without alteration in smooth muscle cell markers. The absence of predisposing conditions (hypertension, smoking, genetic disorders) led us to explore a potential link between fostamatinib and AAD. Experimental data suggest that SYK inhibition exacerbates aortic wall vulnerability, and off‐target effects of fostamatin

Immune thrombocytopenia (ITP) is a rare autoimmune disorder characterized by platelet destruction. While most patients respond to first‐ or second‐line therapies, a small subset is multirefractory. Fostamatinib , an oral spleen tyrosine kinase ( SYK ) inhibitor, is a therapeutic option in these cases. We report a case of an acute aortic dissection (AAD) occurring in a patient without traditional cardiovascular risk factors, 3 months after fostamatinib initiation. A 70‐year‐old woman with a 30‐year history of ITP, unresponsive to multiple therapies, was treated with fostamatinib and achieved a complete haematologic response. She presented with sudden chest pain and was diagnosed with Stanford A (DeBakey type 1) AAD requiring emergency surgery. Histological analysis of her aortic tissue showed a 25% reduction in phosphorylated SYK expression compared to a control sample, without alteration in smooth muscle cell markers. The absence of predisposing conditions (hypertension, smoking, genetic disorders) led us to explore a potential link between fostamatinib and AAD. Experimental data suggest that SYK inhibition exacerbates aortic wall vulnerability, and off‐target effects of fostamatin

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