Congenital Neutropenia and Rare Functional Phagocyte Disorders in Children.

Child Congenital Bone Marrow Failure Syndromes / genetics Granulocyte Colony-Stimulating Factor / therapeutic use Hematopoietic Stem Cell Transplantation / methods Humans Leukemia / genetics Myelodysplastic Syndromes / genetics Neutropenia / congenital Neutrophils / immunology Phagocytes / immunology Rare Diseases / genetics
ELANE Granulocyte colony-stimulating factor (G-CSF) Neutropenia Oxidative burst Phagocytes

Journal

Hematology/oncology clinics of North America
ISSN: 1558-1977
Titre abrégé: Hematol Oncol Clin North Am
Pays: United States
ID NLM: 8709473

Informations de publication

Date de publication:
06 2019
Historique:
entrez: 30 4 2019
pubmed: 30 4 2019
medline: 27 5 2020
Statut: ppublish

Résumé

Both profound neutropenia and functional phagocyte disorders render patients susceptible to recurrent, unusual, and/or life-threatening infections. Many disorders also have nonhematologic manifestations and a substantial risk of leukemogenesis. Diagnosis relies on clinical suspicion and interrogation of the complete blood count with differential/bone marrow examination coupled with immunologic and genetic analyses. Treatment of the quantitative neutrophil disorders depends on granulocyte colony-stimulating factor, whereas management of functional phagocyte disease is reliant on antimicrobials and/or targeted therapies. Hematopoietic stem cell transplant remains the only curative option for most disorders but is not used on a routine basis.

Identifiants

DOI: 10.1016/j.hoc.2019.01.004 PMID: 31030818
pubmed: 31030818
pii: S0889-8588(19)30020-6
doi: 10.1016/j.hoc.2019.01.004
pii:
doi:

Substances chimiques

Granulocyte Colony-Stimulating Factor 143011-72-7

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

533-551

Informations de copyright

Copyright © 2019 Elsevier Inc. All rights reserved.

Auteurs

Kelly Walkovich (K)

Pediatric Hematology/Oncology, Department of Pediatrics, University of Michigan Medical School, 1500 E. Medical Center Drive, D4202 Medical Professional Building, SPC 5718, Ann Arbor, MI 48109-5718, USA. Electronic address: kwalkovi@med.umich.edu.

James A Connelly (JA)

Pediatric Hematopoietic Stem Cell Transplant, Department of Pediatrics, Vanderbilt University Medical Center, 2220 Pierce Avenue, 397 PRB, Nashville, TN 37232-6310, USA.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies néonatales › Insuffisances médullaires congénitales › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Facteurs biologiques › Protéines et peptides de signalisation intercellulaire › Cytokines › Facteurs de croissance hématopoïétique › Facteurs de stimulation des colonies › Facteur de stimulation des colonies de granulocytes › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Procédures de chirurgie opératoire › Transplantation › Transplantation cellulaire › Transplantation de cellules souches › Transplantation de cellules souches hématopoïétiques › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Tumeurs › Tumeurs par type histologique › Leucémies › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Hémopathies et maladies lymphatiques › Hémopathies › Maladies de la moelle osseuse › Syndromes myélodysplasiques › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Hémopathies et maladies lymphatiques › Hémopathies › Troubles leucocytaires › Leucopénie › Agranulocytose › Neutropénie › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système immunitaire › Phagocytes › Granulocytes neutrophiles › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système immunitaire › Phagocytes › Congenital Neutropenia and Rare Functional...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Caractéristiques de la maladie › Maladies rares › Congenital Neutropenia and Rare Functional...