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

Juliette Tennenbaum (1) , M. Groh (2) , L. Venditti (3) , France Campos-Gazeau (4) , Emilie Chalayer , T. de Broucker (5) , M. Hamidou (6, 7) , M. Hunault (8) , A. Lyoubi , R. Meunier (9) , Thierry Muron , D. Sène (10) , B. Slama (11) , C. Guidoux , Guillaume Lefevre (12) , Jean-Emmannuel Kahn (12) , C. Denier , J. Rohmer (2)

1 Service de neurologie [Le Kremlin Bicêtre]
2 Hôpital Foch [Suresnes]
3 Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
4 Centre Hospitalier de Haguenau
5 Hôpital Delafontaine
6 Service de médecine interne [Nantes]
7 CHU Nantes - Centre Hospitalier Universitaire de Nantes
8 CRCNA - Centre de Recherche en Cancérologie Nantes-Angers
9 Centre Hospitalier Libourne
10 Hôpital Lariboisière-Fernand-Widal [APHP]
11 Centre Hospitalier Henri Duffaut (Avignon)
12 LIRIC - Lille Inflammation Research International Center - U 995

Background and Purpose: 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. Methods: 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. Results: 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). Conclusions: 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.

Mots clés: eosinophils; imatinib mesylate; leukemia; stroke

Domaines: Sciences du Vivant [q-bio]

Juliette Tennenbaum, M. Groh, L. Venditti, France Campos-Gazeau, Emilie Chalayer, et al.. FIP1L1-PDGFRA-Associated Hypereosinophilic Syndrome as a Treatable Cause of Watershed Infarction.. Stroke, 2021, Stroke, pp.605-609. ⟨10.1161/STROKEAHA.121.034191⟩. ⟨hal-04486058⟩