Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country.
Colombia
Hemophagocytic
developing country
lymphohistiocytosis
pediatrics
Journal
BMC pediatrics
ISSN: 1471-2431
Titre abrégé: BMC Pediatr
Pays: England
ID NLM: 100967804
Informations de publication
Date de publication:
18 09 2021
18 09 2021
Historique:
received:
17
11
2020
accepted:
06
09
2021
entrez:
19
9
2021
pubmed:
20
9
2021
medline:
26
10
2021
Statut:
epublish
Résumé
Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host's inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries. Retrospective, descriptive study of pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete an electronic database and a secondary, descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee. Twenty-one patients were included. 52.4 % of the population was male with a median age of 9.3 years [IQR (3.0-13.7 years)]. More than half of patients (66.6 %) had viral disease at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3 %) and dengue (14.3 %). Three patients had confirmed gene mutations (G6PC3, XIAP, and UNC13D). 95 % of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with intravenous immunoglobulin (IVIG) and/or systemic steroids, while the other half received the complete protocol including etoposide and cyclosporine. More than three-fourths (76.2 %) required admission to an ICU with a median stay of 14 days [IQR (11-37 days)] and a median hospital stay of 30 days [IQR (18-93 days)]. 14.3 % (n = 3) of patients died. HLH is a complex disease that requires multidisciplinary management with secondary HLH due to EBV infection being a common cause. There is increasing awareness of HLH diagnosis in developing countries such as Colombia which can offer earlier treatment options and better outcomes.
Sections du résumé
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host's inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries.
METHODS
Retrospective, descriptive study of pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete an electronic database and a secondary, descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee.
RESULTS
Twenty-one patients were included. 52.4 % of the population was male with a median age of 9.3 years [IQR (3.0-13.7 years)]. More than half of patients (66.6 %) had viral disease at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3 %) and dengue (14.3 %). Three patients had confirmed gene mutations (G6PC3, XIAP, and UNC13D). 95 % of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with intravenous immunoglobulin (IVIG) and/or systemic steroids, while the other half received the complete protocol including etoposide and cyclosporine. More than three-fourths (76.2 %) required admission to an ICU with a median stay of 14 days [IQR (11-37 days)] and a median hospital stay of 30 days [IQR (18-93 days)]. 14.3 % (n = 3) of patients died.
CONCLUSIONS
HLH is a complex disease that requires multidisciplinary management with secondary HLH due to EBV infection being a common cause. There is increasing awareness of HLH diagnosis in developing countries such as Colombia which can offer earlier treatment options and better outcomes.
Identifiants
pubmed: 34537050
doi: 10.1186/s12887-021-02879-7
pii: 10.1186/s12887-021-02879-7
pmc: PMC8449481
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
411Informations de copyright
© 2021. The Author(s).
Références
N Engl J Med. 2020 May 7;382(19):1811-1822
pubmed: 32374962
Haematologica. 2020 May;105(5):e210-e212
pubmed: 31515353
Indian J Clin Biochem. 2018 Jan;33(1):108-112
pubmed: 29371779
PLoS One. 2014 Apr 07;9(4):e94024
pubmed: 24710079
Am J Clin Pathol. 2016 Jun;145(6):862-70
pubmed: 27298397
Nat Rev Clin Oncol. 2010 Jul;7(7):415-20
pubmed: 20404855
Curr Opin Pediatr. 2012 Jun;24(3):400-6
pubmed: 22525720
Pediatr Blood Cancer. 2007 Feb;48(2):124-31
pubmed: 16937360
Blood. 2005 Feb 1;105(3):994-6
pubmed: 15494422
Clin Lymphoma Myeloma Leuk. 2017 Jul;17S:S105-S110
pubmed: 28760295
Eur J Pediatr. 2007 Feb;166(2):95-109
pubmed: 17151879
Front Immunol. 2018 Jan 08;8:1902
pubmed: 29358936
Blood. 2011 Oct 13;118(15):4041-52
pubmed: 21828139
Blood. 2011 Oct 27;118(17):4577-84
pubmed: 21900192
J Clin Pathol. 1987 Dec;40(12):1413-7
pubmed: 3323248
Rev Med Virol. 2018 Nov;28(6):e2005
pubmed: 30109914
J Blood Med. 2014 Jun 12;5:69-86
pubmed: 24966707
Semin Oncol. 1991 Feb;18(1):29-33
pubmed: 1992521
Hematol Oncol Clin North Am. 1998 Apr;12(2):435-44
pubmed: 9561911
Acta Paediatr Scand. 1991 Apr;80(4):428-35
pubmed: 2058392
Pediatr Clin North Am. 2012 Apr;59(2):329-44
pubmed: 22560573
Hematol Oncol Stem Cell Ther. 2019 Sep;12(3):166-170
pubmed: 28834694
Indian J Pediatr. 2016 May;83(5):434-43
pubmed: 26872683
Blood Rev. 2014 Jul;28(4):135-42
pubmed: 24792320
Case Rep Pediatr. 2018 Nov 22;2018:6236270
pubmed: 30595935
N Engl J Med. 2010 May 27;362(21):1993-2000
pubmed: 20505178
Pediatr Blood Cancer. 2008 Jun;50(6):1227-35
pubmed: 18085676
Blood Rev. 2016 Nov;30(6):411-420
pubmed: 27238576
Curr Opin Pediatr. 2012 Feb;24(1):9-15
pubmed: 22189397