Clinical manifestations and outcomes of pediatric chronic neutropenia

Wan, Chunli; Yu, Hsin-Hui; Lu, Meng‐Yao; Lee, Jyh‐Hong; Wang, Li‐Chieh; Lin, Yu-Tsan; Yang, Yao‐Hsu; Chiang, Bor‐Luen

doi:10.1016/j.jfma.2010.12.003

Cited by 19 publications

(26 citation statements)

References 34 publications

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“…in severe congenital neutropenia (sCN), sepsis is a potential cause of death with a cumulative risk assessed at 15% after 20 years of observation, 1,2 despite granulocyte-colony stimulating factor therapy (G-Csf). Conversely, autoimmune neutropenia (AN) and idiopathic neutropenia (iN) patients are reported to have milder infections [3][4][5][6] and sometimes their early occurrence or occurrence in unusual sites (skin, mouth, perianal region) can lead to the diagnosis of neutropenia. 1 epidemiology of infections and detailed description of type and site of infections in both congenital and acquired neutropenia are lacking.…”

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confidence: 99%

“…5 previous studies suggested that respiratory carriage of K. kingae is a prerequisite for distant infection of joint and bone. [6][7][8] the oropharyngeal carriage of K. kingae does not necessary imply the subsequent development of an invasive infection, and the probability for asymptomatic children carrying K. kingae to develop an osteoarticular infection is lower than 1%. 9 Density of oropharyngeal carriage may be a risk factor in the pathogenesis of invasive K. kingae infections, as it is the case with many other pathogens present in oropharyngeal flora, such as Streptococcus pneumoniae or Haemophilus influenzae.…”

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confidence: 99%

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Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Fioredda

Calvillo

Burlando

et al. 2013

Pediatric Infectious Disease Journal

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We describe the incidence and characteristics of infections in children with severe congenital neutropenia (SCN), autoimmune neutropenia (AN) and idiopathic neutropenia (IN). Data extracted from the Italian Neutropenia Registry on 73 patients with 108 episodes of infections were collected from 2000 to 2009. All SCN patients with SCN and one third of AN and IN experienced at least 1 infectious episode, equating to 5.7 infections/patient in SCN and approximately 0.6 in AN and IN. The rate of infections before diagnosis of neutropenia was 6.35/1000 patient-days at risk in SCN, 0.48 in AN and 0.71 in IN (P < 0.001) and significantly decreased after diagnosis. Skin and subcutaneous abscesses and cellulitis were the most frequent types of infection encountered, followed by pneumonia. Infections are an important clinical issue in the management of neutropenic patients, even in those considered at lower risk.

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confidence: 99%

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confidence: 99%

Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Fioredda

Calvillo

Burlando

et al. 2013

Pediatric Infectious Disease Journal

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“…4,6,10 CIN was found to be caused by one of these etiologies in a similar proportion of our patients (34.0% to 68.9%) compared with previously studied cohorts. 7,11,12,15 There were differences in sex and the rate of recovery from neutropenia between the CIN and CoN groups. These results are similar to those of other studies, reporting that CIN and AIN mainly occur in females.…”

Section: Discussionmentioning

confidence: 95%

“…2,3,10 The CoN ratio in the CN patients in this study (45.1%) is higher compared with previous reports (9.0% to 24.1%). 7,11,12 The results of demographic surveys have shown that consanguineous marriages account for 22% of the total marriages in Turkey. 13 Thus, the high CoN ratio in CN patients may be due to high levels of consanguineous marriages in our country.…”

Section: Discussionmentioning

confidence: 99%

Chronic Neutropenia in Childhood

Karapınar

Oymak

et al. 2016

Journal of Pediatric Hematology/Oncology

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Chronic neutropenia (CN) is defined as neutropenia that persists for >3 months; it is caused by a heterogeneous group of diseases in children. The aim of the present study was to evaluate the significance and clinical manifestations of CN in children at a single children's hospital. Between October 2004 and April 2014, CN patient data were evaluated retrospectively. Thirty-one patients were assessed in this study. Thirteen of them (41.9%) were younger than 12 months of age at initial diagnosis. There was no difference in the absolute number of neutrophils at diagnosis between the children aged younger than 12 months and those aged 12 months and older at CN onset. Twenty-two of the patients (70.9%) were diagnosed during treatment for acute infections. A total of 11 patients (35.5%) were hospitalized because of recurrent infections. Most of the recurrent infections occurred in the lungs (81.8%). Congenital neutropenia (CoN) was identified in 14 patients (45.1%). Eight of 14 patients (57.1%) required granulocyte-colony stimulating factor treatment, and none of them experienced adverse effects from this treatment. Fifteen patients (48.3%) were diagnosed with idiopathic neutropenia. Comparison between the idiopathic and CoN groups revealed no differences in age, the absolute number of neutrophils, or the presence of infection at diagnosis; however, differences were detected in sex and the rate of spontaneous recovery from neutropenia. Ten of the patients (32.2%) experienced spontaneous recovery from neutropenia during a follow-up period of 7 to 52 months. In current study, we found a higher CoN ratio in the CN patients compared with previous reports, which may be due to the high rate of consanguineous marriages in our country. However, the finding of CN requires several laboratory investigations, prolonged follow-up, and advanced molecular analysis, and its etiology can remain idiopathic.

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“…In a study from Taiwan, 29 pediatric patients were found to have chronic neutropenia (7 congenital, 7 autoimmune, and 15 idiopathic). 6 According to the French National Registry of Primary Immunodeficiency Diseases, congenital neutropenias occur in ~6 out of 1 000 000. 7 This is likely to be an underestimate, as genetic disorders such as Shwachman-Diamond syndrome (SDS), GATA2 deficiency, and dyskeratosis congenita are being better recognized and genetically confirmed.…”

Section: Neutropenia: Definition Epidemiology and Complicationsmentioning

confidence: 99%

New monogenic disorders identify more pathways to neutropenia: from the clinic to next-generation sequencing

Corey

Oyarbide

2017

Hematology

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Neutrophils are the most common type of leukocyte in human circulating blood and constitute one of the chief mediators for innate immunity. Defined as a reduction from a normal distribution of values, neutropenia results from a number of congenital and acquired conditions. Neutropenia may be insignificant, temporary, or associated with a chronic condition with or without a vulnerability to life-threatening infections. As an inherited bone marrow failure syndrome, neutropenia may be associated with transformation to myeloid malignancy. Recognition of an inherited bone marrow failure syndrome may be delayed into adulthood. The list of monogenic neutropenia disorders is growing, heterogeneous, and bewildering. Furthermore, greater knowledge of immune-mediated and drug-related causes makes the diagnosis and management of neutropenia challenging. Recognition of syndromic presentations and especially the introduction of next-generation sequencing are improving the accuracy and expediency of diagnosis as well as their clinical management. Furthermore, identification of monogenic neutropenia disorders is shedding light on the molecular mechanisms of granulopoiesis and myeloid malignancies.

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Clinical manifestations and outcomes of pediatric chronic neutropenia

Cited by 19 publications

References 34 publications

Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Chronic Neutropenia in Childhood

New monogenic disorders identify more pathways to neutropenia: from the clinic to next-generation sequencing

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