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What is the place of hematopoietic stem cell transplantation in the management of cerebral vasculopathy in children with sickle cell anemia?
Author(s) -
Françoise Bernaudin
Publication year - 2020
Publication title -
hematology/oncology and stem cell therapy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.666
H-Index - 22
eISSN - 1658-3876
pISSN - 2589-0646
DOI - 10.1016/j.hemonc.2019.12.002
Subject(s) - medicine , sickle cell anemia , transcranial doppler , hematopoietic stem cell transplantation , sibling , anemia , transplantation , stem cell , pediatrics , complication , disease , psychology , developmental psychology , biology , genetics
Cerebral vasculopathy is the most severe complication affecting children with sickle cell anemia. Significant progress has been made in the management of sickle cell anemia cerebral vasculopathy, including early transcranial Doppler screening, chronic transfusion, andhydroxyurea. Nevertheless, for patients with a potential matched-sibling donor (MSD), stem cell transplantation (SCT) is now the treatment offering the best cerebral vasculopathy outcome. In the absence of MSD,alternative SCT should be recommended only in those with worsening cerebral vasculopathy despite standard treatments, and should be limited to related haplo-identical SCT undertaken in controlled studies.

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