• Ugo Boccadifuoco, Geoffrey Cheminet, Benjamin Morino, and Jean-Benoît Arlet.
• Service de médecine interne, centre national de référence des syndromes drépanocytaires majeurs de l'adulte, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France.
• Rev Med Interne. 2025 Jan 7.

IntroductionExtramedullary hematopoiesis (EMH) is very rarely described during sickle cell disease (SCD). Our aim was to describe six cases of EMH occurring in adult SCD patients and to conduct a literature review.MethodsRetrospective, descriptive, and monocentric study, identifying all cases of EMH recorded in our cohort of adult SCD patients, up to April 2024. A literature review via PubMed included thirty-five articles (44 patients).ResultsSix patients (4 men, 83.3% with SS genotype [n=5], 1 SC), with a median age of 22 (range 12-64) years at the time of EMH diagnosis were included. Four patients (66.7%) had an aseptic osteonecrosis of the hip. The localization of EMH varied: paravertebral (n=3), peri-articular in the hip (n=1), adrenal (n=1), hepatic (n=1), splenic (n=1) and was similar to the localizations reported in the literature. EMH was symptomatic at diagnosis in half of the cases. The diagnosis was established by histology (n=3/3) and/or typic magnetic resonance imaging (MRI) (n=4/4). The median baseline hemoglobin was 9.1 (extremes 5.8-10.9) g/dL. A watch-and-wait approach was primarily observed.ConclusionEMH in SCD patients appears to be rare, with varied localizations. Its diagnosis is made with MRI and/or biopsy, and its treatment is not consensual.Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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