FIP1L1-PDGFRA-Associated Hypereosinophilic Syndrome as a Treatable Cause of Watershed Infarction

• Published in: Stroke (1970) Vol. 52; no. 10; pp. e605 - e609
• Main Authors: Tennenbaum, Juliette, Groh, Matthieu, Venditti, Laura, Campos-Gazeau, France, Chalayer, Emilie, De Broucker, Thomas, Hamidou, Mohamed, Hunault, Mathilde, Lyoubi, Aicha, Meunier, Raphaëlle, Muron, Thierry, Sène, Damien, Slama, Borhane, Guidoux, Céline, Lefèvre, Guillaume, Kahn, Jean-Emmanuel, Denier, Christian, Rohmer, Julien
• Format: Journal Article
• Language: English
• Published: United States American Heart Association 01.10.2021
• Series: Stroke
• Subjects: Adult; Brain - diagnostic imaging; Cerebral Infarction - diagnostic imaging; Cerebral Infarction - etiology; Cerebral Infarction - therapy; Coronary Thrombosis - complications; Female; Follow-Up Studies; Humans; Hypereosinophilic Syndrome - complications; Hypereosinophilic Syndrome - diagnostic imaging; Hypereosinophilic Syndrome - therapy; Imatinib Mesylate - therapeutic use; Ischemic Stroke - diagnostic imaging; Ischemic Stroke - genetics; Ischemic Stroke - therapy; Life Sciences; Male; Middle Aged; mRNA Cleavage and Polyadenylation Factors - genetics; Oncogene Proteins, Fusion - genetics; Receptor, Platelet-Derived Growth Factor alpha - genetics; Recurrence; Retrospective Studies; Treatment Outcome; leukemia; eosinophils; imatinib mesylate; stroke
• DOI: 10.1161/STROKEAHA.121.034191
• ISSN: 0039-2499

Ischemic stroke has been reported in various conditions associated with eosinophilia. FIP1L1-PDGFRA fusion ([Fip1-like 1-platelet-derived growth factor receptor alpha]; F/P) leads to the proliferation of the eosinophilic lineage and thus to a clonal hypereosinophilic syndrome that is highly responsive to imatinib. We previously reported on a nationwide retrospective study of 151 patients with F/P-associated clonal hypereosinophilic syndrome. Patients from this cohort with a clinical history of ischemic stroke (as well as 2 additional cases) were further analyzed to better define their clinical picture and outcomes. Sixteen male patients (median age, 51 [43–59] years) with low-to-intermediate cardiovascular risk were included. Median National Institutes of Health Stroke Scale was 4 (range, 1–6). Most cerebral imaging disclosed multiple bilateral infarctions of watershed distribution (69%). Despite frequent cardiac involvement (50%), cardiac thrombus was evidenced in a single patient and, according to the TOAST classification (Trial of ORG 10172 in Acute Stroke Treatment), 62.5% of strokes were presumably of undetermined etiology. Among the 15 patients treated with imatinib, and after a median follow-up of 4.5 years, stroke recurred in only 3 patients (consisting of either cardio embolic or hemorrhagic events, unrelated to the first episode). F/P+ clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.