David Calvet1, Françoise Bernaudin1, Antoine Gueguen1, Hassan Hosseini1, Anoosha Habibi1, Frédéric Galactéros1, Pablo Bartolucci2. 1. From the Department of Neurology Hôpital Sainte-Anne, Université Paris-Descartes, INSERM 894, DHU Neurovasc-Paris Sorbonne, Paris, France (D.C.); Pediatrics, Referral Center for Sickle Cell Disease, Centre Hospitalier Intercommunal, Créteil, France (F.B.); Fondation Ophtalmologique A. de Rothschild, Paris, France (A.G.); Service de Neurologie, Hôpital Henri-Mondor, UPEC, Créteil, France (H.H.); Department of Neurology Sickle cell referral center, médecine interne, Hôpital Henri-Mondor, UPEC, Créteil, France (A.H, F.G., P.B.); and INSERM U955, team 2, Laboratoire d'Excellence Grex, Créteil, France (A.H, F.G., P.B.). 2. From the Department of Neurology Hôpital Sainte-Anne, Université Paris-Descartes, INSERM 894, DHU Neurovasc-Paris Sorbonne, Paris, France (D.C.); Pediatrics, Referral Center for Sickle Cell Disease, Centre Hospitalier Intercommunal, Créteil, France (F.B.); Fondation Ophtalmologique A. de Rothschild, Paris, France (A.G.); Service de Neurologie, Hôpital Henri-Mondor, UPEC, Créteil, France (H.H.); Department of Neurology Sickle cell referral center, médecine interne, Hôpital Henri-Mondor, UPEC, Créteil, France (A.H, F.G., P.B.); and INSERM U955, team 2, Laboratoire d'Excellence Grex, Créteil, France (A.H, F.G., P.B.). pablo.bartolucci@hmn.aphp.fr.
Abstract
BACKGROUND AND PURPOSE: There is little evidence about characteristics of ischemic stroke (IS) occurring in adults with sickle-cell disease (SCD). The objective of this study was to assess characteristics of first-ever IS in adults with SCD and to assess whether they differ from those occurring in child patients with SCD. METHODS: Adult and child individuals with SCD who had a first-ever IS were identified from cohorts of patients followed up in an adult and a child sickle cell referral center. Mechanisms of IS were determined by consensus meeting from all available explorations using the following predefined classification: Vasculopathy, cardioembolism, other defined cause, and undetermined. Treatment and stroke recurrences were recorded from prospective follow-up performed in the referral centers. RESULTS: Twenty-nine adults and 26 children had a first-ever IS; mean age (SD) was 7.1 (4.3) and 32.3 (11.6), respectively. With regard to IS mechanism, vasculopathy was less often the cause of IS in adults (12/29, 41%) than in children (24/26, 92%; P<0.001). Other causes of IS in adults were cardioembolism in 7, antiphospholipid syndrome in 1, toxic (cocaine) in 1, and undetermined in 8. Adults with SCD had a higher risk of recurrent stroke (23.1% [7.0-39.2] at 5 years) compared with children (1 recurrence only; P log rank=0.046) despite exchange-blood transfusion in patients with vasculopathy. CONCLUSIONS: First-ever IS occurring in adults with SCD has specificities that justify further studies conducted in adults with SCD to improve understanding and management.
BACKGROUND AND PURPOSE: There is little evidence about characteristics of ischemic stroke (IS) occurring in adults with sickle-cell disease (SCD). The objective of this study was to assess characteristics of first-ever IS in adults with SCD and to assess whether they differ from those occurring in childpatients with SCD. METHODS: Adult and child individuals with SCD who had a first-ever IS were identified from cohorts of patients followed up in an adult and a child sickle cell referral center. Mechanisms of IS were determined by consensus meeting from all available explorations using the following predefined classification: Vasculopathy, cardioembolism, other defined cause, and undetermined. Treatment and stroke recurrences were recorded from prospective follow-up performed in the referral centers. RESULTS: Twenty-nine adults and 26 children had a first-ever IS; mean age (SD) was 7.1 (4.3) and 32.3 (11.6), respectively. With regard to IS mechanism, vasculopathy was less often the cause of IS in adults (12/29, 41%) than in children (24/26, 92%; P<0.001). Other causes of IS in adults were cardioembolism in 7, antiphospholipid syndrome in 1, toxic (cocaine) in 1, and undetermined in 8. Adults with SCD had a higher risk of recurrent stroke (23.1% [7.0-39.2] at 5 years) compared with children (1 recurrence only; P log rank=0.046) despite exchange-blood transfusion in patients with vasculopathy. CONCLUSIONS: First-ever IS occurring in adults with SCD has specificities that justify further studies conducted in adults with SCD to improve understanding and management.
Authors: Michael M Dowling; Charles T Quinn; Claudio Ramaciotti; Julie Kanter; Ifeyinwa Osunkwo; Baba Inusa; Rathi Iyer; Janet L Kwiatkowski; Clarissa Johnson; Melissa Rhodes; William Owen; John J Strouse; Julie A Panepinto; Lynne Neumayr; Sharada Sarnaik; Patricia A Plumb; Nomazulu Dlamini; Fenella Kirkham; Linda S Hynan Journal: Br J Haematol Date: 2016-10-21 Impact factor: 6.998
Authors: Hanne Stotesbury; Jamie M Kawadler; Patrick W Hales; Dawn E Saunders; Christopher A Clark; Fenella J Kirkham Journal: Front Neurol Date: 2019-08-13 Impact factor: 4.003