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Reduction of CFU-GM and circulating hematopoietic progenitors in a subgroup of children with chronic neutropenia associated with severe infections and delayed recovery.
Timeus, Fabio; Crescenzio, Nicoletta; Foglia, Luiselda; Doria, Alessandra; Stillitano, Maria Giuseppina; Garelli, Emanuela; Mazzone, Raffaela; Vivalda, Laura; Vallero, Stefano; Ramenghi, Ugo; Saracco, Paola.
Afiliação
  • Timeus F; Pediatric Onco-Hematology, Regina Margherita Children Hospital, Turin, Italy.
  • Crescenzio N; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Foglia L; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Doria A; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Stillitano MG; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Garelli E; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Mazzone R; Biochemistry Laboratory, Regina Margherita Children Hospital, Turin, Italy.
  • Vivalda L; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Vallero S; Pediatric Onco-Hematology, Regina Margherita Children Hospital, Turin, Italy.
  • Ramenghi U; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
  • Saracco P; Pediatric Hematology, Regina Margherita Children Hospital, University of Turin, Turin, Italy.
PLoS One ; 14(3): e0213782, 2019.
Article em En | MEDLINE | ID: mdl-30870474
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.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Células-Tronco Hematopoéticas / Recuperação de Função Fisiológica / Células Progenitoras de Granulócitos e Macrófagos / Infecções / Neutropenia Tipo de estudo: Etiology_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Ano de publicação: 2019 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Células-Tronco Hematopoéticas / Recuperação de Função Fisiológica / Células Progenitoras de Granulócitos e Macrófagos / Infecções / Neutropenia Tipo de estudo: Etiology_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Ano de publicação: 2019 Tipo de documento: Article