Improved stenosis outcome in stroke‐free sickle cell anemia children after transplantation compared to chronic transfusion

Autor: David Grevent, Florence Missud, Suzanne Verlhac, Annie Kamdem, Lydia Divialle-Doumdo, Charlotte Jubert, Corinne Guitton, Corinne Pondarré, Isabelle Thuret, Flaviu Gabor, Alexandra Gauthier, Cécile Arnaud, Jean-François Chateil, Philippe Petit, Françoise Bernaudin, Mariane de Montalembert, Marie Petras, Valentine Brousse, Catherine Paillard, Monique Elmaleh
Rok vydání: 2020
Předmět:
Zdroj: British Journal of Haematology. 193:188-193
ISSN: 1365-2141
0007-1048
Popis: We report here the 3-year stenosis outcome in 60 stroke-free children with sickle cell anaemia (SCA) and an abnormal transcranial Doppler history, enrolled in the DREPAGREFFE trial, which compared stem cell transplantation (SCT) with standard-care (chronic transfusion for 1-year minimum). Twenty-eight patients with matched sibling donors were transplanted, while 32 remained on standard-care. Stenosis scores were calculated after performing cerebral/cervical 3D time-of-flight magnetic resonance angiography. Fourteen patients had stenosis at enrollment, but only five SCT versus 10 standard-care patients still had stenosis at 3 years. Stenosis scores remained stable on standard-care, but significantly improved after SCT (P = 0·006). No patient developed stenosis after SCT, while two on standard-care did, indicating better stenosis prevention and improved outcome after SCT.
Databáze: OpenAIRE