Reduction of CFU-GM and circulating hematopoietic progenitors in a subgroup of children with chronic neutropenia associated with severe infections and delayed recovery.
Journal
PloS one
ISSN: 1932-6203
Titre abrégé: PLoS One
Pays: United States
ID NLM: 101285081
Informations de publication
Date de publication:
2019
2019
Historique:
received:
17
08
2018
accepted:
01
03
2019
entrez:
15
3
2019
pubmed:
15
3
2019
medline:
18
12
2019
Statut:
epublish
Résumé
Myelopoiesis was evaluated in 66 pediatric patients with chronic neutropenia who were positive for anti-neutrophil antibodies (median age at diagnosis: 11 months, median neutrophil count at diagnosis: 419/μl). Other causes of neutropenia were excluded. Bone marrow morphology, clonogenic tests and/or the peripheral blood CD 34+ cell count, and apoptotic rate were evaluated in 61 patients with neutropenia lasting > 12 months or severe infections. The peripheral blood CD 34+ cell count and apoptotic rate were evaluated in five patients with shorter neutropenia. The median follow-up time was 29 months (range 7-180 months). Forty-seven patients (71.2%) had a spontaneous recovery after 7-180 months (median 29 months). The group of patients younger than 24 months at diagnosis (n = 50) had a higher probability of recovery (40/50 vs. 7/16 χ2 p<0.01) with a shorter period of neutropenia (median 26 versus 47 months, Kaplan-Meier analysis p = 0.001). The colony-forming units-granulocyte-macrophage (CFU-GM) were significantly decreased in 26/35 patients (74%) evaluated for clonogenic tests. All patients with normal CFU-GM recovered (9/9 patients); whereas, neutropenia persisted in 12/26 patients with reduced CFU-GM (46%, Pearson χ2 p = 0.02). In 36/55 (65%) patients evaluated by flow cytometry we observed reduced circulating CD34+ cells compared with controls of the same age. An increase in the circulating CD34+ cell apoptotic rate was observed in 28/55 patients (51%). Infections requiring hospitalization were observed in 9/18 (50%; Pearson χ2, p = 0.03) patients with both decreased circulating CD34+ cells and increased CD34+ apoptotic rates. In the group aged < 24 months, we observed a significant correlation between the persistence of neutropenia and decreased circulating CD34+ cells (Pearson χ2 p = 0.008). In conclusion, reduced CFU-GM and circulating hematopoietic progenitors were observed in a subgroup of children with chronic neutropenia who were positive for anti-neutrophil antibodies and had a higher incidence of severe infections and delayed spontaneous remission.
Identifiants
pubmed: 30870474
doi: 10.1371/journal.pone.0213782
pii: PONE-D-18-24311
pmc: PMC6417780
doi:
Substances chimiques
Antigens, CD34
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e0213782Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
Références
J Clin Pathol. 1992 May;45(5):431-4
pubmed: 1597523
Pediatr Allergy Immunol. 2011 Aug;22(5):494-6
pubmed: 21771084
Exp Hematol. 2005 May;33(5):597-604
pubmed: 15850838
Vox Sang. 2013 Oct;105(3):259-69
pubmed: 23663230
Transfusion. 2009 May;49(5):1003-6
pubmed: 19210322
Am J Hematol. 1985 Nov;20(3):223-34
pubmed: 3877459
Am J Hematol. 2015 Dec;90(12):E221-2
pubmed: 26361081
Vox Sang. 1990;59(4):251-6
pubmed: 1981408
Acta Paediatr. 2003 Nov;92(11):1277-83
pubmed: 14696847
Transfusion. 1997 Sep;37(9):977-83
pubmed: 9308648
Br J Haematol. 1978 Jul;39(3):339-50
pubmed: 359031
Vox Sang. 2005 Jan;88(1):52-9
pubmed: 15663723
Am J Hematol. 2012 Feb;87(2):238-43
pubmed: 22213173
J Hematother. 1996 Jun;5(3):213-26
pubmed: 8817388
Blood. 1999 Sep 1;94(5):1797-802
pubmed: 10477706
Blood. 1994 Jul 15;84(2):625-31
pubmed: 7517722
Pediatr Clin North Am. 1996 Jun;43(3):773-92
pubmed: 8649909
J Immunol Methods. 1999 Dec 17;232(1-2):211-29
pubmed: 10618522
Blood. 1998 Jan 1;91(1):181-6
pubmed: 9414283
Blood. 1987 Feb;69(2):529-36
pubmed: 3801668
Pediatr Blood Cancer. 2011 Jul 15;57(1):10-7
pubmed: 21448998
Curr Opin Allergy Clin Immunol. 2004 Dec;4(6):513-21
pubmed: 15640692
Pediatr Allergy Immunol. 2014 May;25(3):283-9
pubmed: 24325465