Autoimmune and other acquired neutropenias

Newburger, Peter E.

doi:10.1182/asheducation-2016.1.38

Cited by 45 publications

(60 citation statements)

References 33 publications

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“…BEN was considered as an alternative diagnosis as these patients demonstrated persistent neutropenia and their records did not indicate the presence of any infection or malignancy, thereby fulfilling the criteria for the diagnosis [ 19 ]. BEN is a clinical diagnosis [ 20 , 21 ]. However, one study found a significant association between persistent neutropenia in patients of African ancestry and polymorphism in the Duffy antigen receptor for chemokines (SNP rs2814778 at chromosome 1q23.2).…”

Section: Discussionmentioning

confidence: 99%

“…Benign ethnic neutropenia (BEN) is a relatively common condition throughout the Middle East, especially among those of African descent [ 19 ]. It is a clinical diagnosis based on persistent neutropenia with normal levels of other white blood cell lines, as well as the absence of secondary causes of neutropenia or hematological disorders [ 20 – 23 ]. There are many reports of the prevalence of BEN in Arab populations.…”

Section: Introductionmentioning

confidence: 99%

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White blood cell subpopulation changes and prevalence of neutropenia among Arab diabetic patients attending Dasman Diabetes Institute in Kuwait

et al. 2018

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BackgroundThe effects of diabetes mellitus on the differential white blood cell count are not widely studied in the Arab populations. The objective of this cross-sectional, retrospective study is to assess the influence of chronic diabetes mellitus on white blood cell counts, absolute neutrophil (ANC) and lymphocyte counts (ALC) as well as the prevalence of benign ethnic neutropenia among Arabs attending the Dasman Diabetes Institute (DDI) in Kuwait.Methods and findings1,580 out of 5,200 patients registered in the DDI database qualified for our study. Age, gender, HbA1c and creatinine levels, estimated glomerular filtration rate as well as average WBC, ANC and ALC levels, presence of diabetes-associated complications and anti-diabetic medications were analyzed. Our results showed the mean value of the WBC was 7.6 ± 1.93 x 109/L (95% CI: 2.95–17.15). The mean ANC was 4.3 x 109/L (95% CI: 0.97–10.40) and mean ALC was 2.5 x 109/L (95% CI: 0.29–10.80). Neutropenia (ANC: <1.5 x 109/L) was detected in fifteen patients (0.94%). Six patients (0.4%) fulfilled the definition of lymphopenia (ALC < 1 x109/L). Patients with an HbA1c ≥ 7% and those taking at least 3 anti-diabetic medications showed higher values for ANC and ALC. Patients with diabetes-associated neuropathy or nephropathy displayed higher mean ANC values. Our study was limited by overrepresentation of patients over 50 years old compared to those under 50 as well as selection bias given its retrospective nature.ConclusionsOur study showed that patients with poorly controlled diabetes displayed higher ANC and ALC levels. In addition, patients with DM-associated complications showed higher ANC levels. This finding would suggest that DM exerts a pro-inflammatory influence on differential WBC counts. Our study also showed that the prevalence of benign ethnic neutropenia was lower than previously reported in other studies.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

White blood cell subpopulation changes and prevalence of neutropenia among Arab diabetic patients attending Dasman Diabetes Institute in Kuwait

et al. 2018

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“…1 Neutropenia in AIN is a result of peripheral destruction of neutrophils due to autoantibody production against antigens on the neutrophil surface, most commonly human neutrophil antigen 1 (HNA-1) on the neutrophil immunoglobulin receptor Fc RIIIb. 8,9 Antibodies often remit within 18 to 24 months from the time of diagnosis, resulting in spontaneous remission. As a consequence, patients with AIN have lower rates of infection due to normal marrow storage pool of neutrophils and rarely develop severe infections.…”

Section: Introductionmentioning

confidence: 99%

The cost of a “benign” condition: Healthcare utilization and infectious outcomes in young children with primary autoimmune neutropenia

Kirk

Grimes

Shelke

et al. 2019

Pediatric Blood & Cancer

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Background Autoimmune neutropenia (AIN) is a common cause of chronic neutropenia in childhood. Despite an expected benign clinical course, many patients undergo extensive evaluation. Data on healthcare utilization and rates of bloodstream infections in young patients with AIN are limited. Methods All patients with a diagnosis code of leukopenia, neutropenia, or AIN followed within the outpatient hematology clinic of a single institution from 2014 to 2016 were identified. Patients aged ≤5 years with absolute neutrophil count (ANC) ≤500/µL persisting for ≥3 months, a clinical diagnosis of AIN, and documented resolution of neutropenia were included. Data on clinical management, including infectious outcomes and emergency center (EC) encounters, were collected. Results Forty‐three patients with AIN (18 male [42%], median age at diagnosis 12 months) met eligibility criteria. Children were followed by hematology for a median duration of 18 (range, 2‐85) months. Diagnostic evaluations were variable. Thirty patients (70%) had ≥ 1 EC encounters for evaluation of isolated fever with a total of 113 EC encounters for the overall cohort. Patients with ANC < 500/µL and isolated fever were admitted for observation, which resulted in 24 hospitalizations in 16 patients. Of 138 blood cultures drawn, two were positive, both later determined to be contaminants. Conclusion At a large tertiary care center, no bloodstream infections were identified in a cohort of 43 children with AIN presenting to the EC for assessment of fever. A less‐intensive, more cost‐effective management paradigm, which continues to prioritize patient safety, among young children with AIN is needed.

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“…Neutrophils are an important part of the body’s defense system and prevent bacterial and fungal infections by phagocytosis and killing foreign invaders. A neutrophil count below 1,500 cells/μL is termed neutropenia, and it becomes clinically significant if the counts fall below 200, especially in immunocompromised states [1]. Hence, neutropenia is alarming to the patient families, as well as certain clinicians, even if it is transient.…”

Section: Introductionmentioning

confidence: 99%

Isolated Chronic and Transient Neutropenia

Singh

Lubana

Dabrowski

2019

Cureus

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Neutrophils are a critical part of the body’s defense system to prevent serious bacterial and fungal infections. Neutropenia is a term which is defined by the absolute neutrophil counts (ANC) < 1,500 cells/µL, and it becomes clinically significant when the level falls below 500 cells/µL. The risk of morbidity and mortality increases considerably when the levels fall below 200. In some ethnicities, the neutropenia is chronic and is frequently seen on routine outpatient visits. On the other hand, transient neutropenia is associated with a transient drop in the neutrophil count and many of the underlying causes are reversible. Patients and their families, as well as some clinicians, express great concern for neutropenia, leading to a multitude of tests and emergency room visits. In this review, we discuss the causes of both chronic and transient neutropenia. Also, we have given special emphasis on the mechanism of neutropenia and management of transient neutropenia.

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Autoimmune and other acquired neutropenias

Cited by 45 publications

References 33 publications

White blood cell subpopulation changes and prevalence of neutropenia among Arab diabetic patients attending Dasman Diabetes Institute in Kuwait

White blood cell subpopulation changes and prevalence of neutropenia among Arab diabetic patients attending Dasman Diabetes Institute in Kuwait

The cost of a “benign” condition: Healthcare utilization and infectious outcomes in young children with primary autoimmune neutropenia

Isolated Chronic and Transient Neutropenia

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