About: HSCT may lower leukemia risk in ELANE neutropenia: a before–after study from the French Severe Congenital Neutropenia Registry

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About: HSCT may lower leukemia risk in ELANE neutropenia: a before–after study from the French Severe Congenital Neutropenia Registry Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : covidontheweb.inria.fr associated with source document(s)

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
title	HSCT may lower leukemia risk in ELANE neutropenia: a before–after study from the French Severe Congenital Neutropenia Registry
Creator	Moshous, Despina Gandemer, Virginie Aladjdi, Nathalie Beaupain, Blandine Bellanne-Chantelot, Christine Bertrand, Yves Dalle, Jean-Hugues De Fontbrune, Sicre De La Tour, Peffault Donadieu, Jean Donadieu, • Dore, Eric Filhon, Bruno Flore, • Galambrun, Claire Nachit, Ouahiba Neven, Benedicte Ouachee, Marie Paillard, Catherine Regis, • Rialland, Fanny Rotulo, Gioacchino
source	PMC
abstract	ELANE neutropenia is associated with myelodysplasia and acute leukemia (MDS–AL), and severe infections. Because the MDS–AL risk has also been shown to be associated with exposure to GCSF, since 2005, in France, patients receiving high daily GCSF doses (>15 μg/kg/day) are eligible for HSCT, in addition to classic indications (MDS–AL or GCSF refractoriness). We analyzed the effect of this policy. Among 144 prospectively followed ELANE-neutropenia patients enrolled in the French Severe Congenital Neutropenia Registry, we defined two groups according to period: “before 2005” for those born before 2005 and followed until 31/12/2004 (1588 person-years); and “after 2005” comprised of those born after 2005 or born before 2005 but followed after 2005 until 31/03/2019 (1327 person-years). Sixteen of our cohort patients underwent HSCT (14 long-term survivors) and six developed MDS–ALs. Six leukemic transformations occurred in the before-2005 group and none after 2005 (respective frequencies 3.8 × 10(–3) vs. 0; P < 0.01), while four HSCTs were done before 2005 and 12 since 2005 (respective HSCT rates increased 2.5 × 10(–3) vs. 9 × 10(–3); P < 0.01). Our results support early HSCT for patients with ELANE mutations who received high GCSF doses, as it might lower the risk of leukemic transformation.
has issue date	2020-01-28(xsd:dateTime)
bibo:doi	10.1038/s41409-020-0800-1
bibo:pmid	31992846
has license	no-cc
sha1sum (hex)	adb62a03ae3affb63e87e211b00927feb7f6e6c4
schema:url	https://doi.org/10.1038/s41409-020-0800-1
resource representing a document's title	HSCT may lower leukemia risk in ELANE neutropenia: a before–after study from the French Severe Congenital Neutropenia Registry
has PubMed Central identifier	PMC7091645
has PubMed identifier	31992846
schema:publication	Bone Marrow Transplant
resource representing a document's body	covid:adb62a03ae3affb63e87e211b00927feb7f6e6c4#body_text
is schema:about of	named entity 'HSCT' named entity 'French' named entity 'policy' named entity '2005' named entity 'acute leukemia' named entity '144' named entity 'DAILY' named entity 'EARLY' named entity 'ACUTE LEUKEMIA' named entity 'RECEIVED' named entity 'HSCT' named entity 'OCCURRED' named entity 'RISK OF' named entity 'SEVERE' named entity 'TRANSFORMATIONS' named entity 'EFFECT' named entity 'YEARS' named entity 'person-years' named entity 'addition' named entity 'neutropenia' named entity 'receiving' named entity 'high' named entity 'early' named entity 'respective' named entity 'refractoriness' named entity 'GCSF' named entity 'ELANE' named entity 'neutropenia' named entity '3.8' named entity 'leukemic' named entity 'neutropenia' named entity 'MDS' named entity 'HSCT' named entity 'clonal' named entity 'cell counts' named entity 'Chimerism' named entity 'ELANE' named entity 'Congenital neutropenia' named entity 'GCSF' named entity 'neutropenia' named entity '10.75' named entity 'mutation' named entity 'acute myeloid leukemia' named entity 'GCSF' named entity 'thrombocytopenia' named entity 'AML' named entity 'Kaplan-Meier method' named entity 'ATG' named entity 'MDS' named entity 'HSCT' named entity 'acute lymphoblastic leukemia' named entity 'PCR' named entity 'fungal infections' named entity 'follow-up' named entity 'anemia' named entity 'survival rates' named entity 'American College of Medical Genetics and Genomics' named entity 'ELANE' named entity 'MDS' named entity 'pituitary'

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