Reference values for T and B lymphocyte subpopulations in Turkish children and adults

Besci, Özge; Başer, Dilek; Öğülür, İsmail; Berberoğlu, Ayşe Cansu; Kıykım, Ayça; Besci, Tolga; Leblebici, Asım; Ellıdokuz, Hülya; Boran, Perran; Özek, Eren; Haklar, Goncagül; Özen, Ahmet; Barış, Safa; Aydıner, Elif Karakoç

doi:10.3906/sag-2010-176

Cited by 18 publications

(13 citation statements)

References 14 publications

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“…Symptoms, previous middle ear infections, sinus infections and allergic diseases, secondhand smoking, and exposure to public areas such as schools and kindergartens were recorded before nasal endoscopic examination. Ig levels, lymphocyte and neutrophil counts, and lymphocyte subsets were analyzed according to the age of the patients [4][5][6]. The patients who had a chest examination CT were divided into two groups: with and without chronic symptoms.…”

Section: Methodsmentioning

confidence: 99%

Chronic Rhinosinusitis in Patients with Primary Immunodeficiency

Karadeniz

Aliyeva

Aydemir

et al. 2022

Int Arch Allergy Immunol

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Introduction: Primary immunodeficiencies are a heterogeneous group of diseases associated with an increased incidence of infections, autoimmunity, autoinflammatory diseases, allergies, and cancer. Rhinosinusitis is one of the most common infections in these patients. In our study, we aimed to determine the presence of chronic rhinosinusitis in our patients with primary immunodeficiency and to investigate the etiology of chronic rhinosinusitis. Methods: Forty-four patients (age range: 4–26 years) diagnosed with primary immunodeficiency were enrolled in our study. Patients were interviewed about the symptoms of chronic rhinosinusitis, and nasal endoscopic examinations were performed prospectively. The results of laboratory tests, medications, skin allergy tests, and the patients’ lung computed tomography were retrospectively recorded from patient files. Results: The distribution of patients’ diagnoses included 38.6% (n = 17) primary antibody deficiencies, 6.6% (n = 3) combined immunodeficiencies, 27.3% (n = 12) combined immunodeficiencies with syndromic features, 6.8% (n = 3) phagocytic disorders, and 20.5% (n = 9) immune dysregulation disorders. There was no significant difference in the frequency of chronic rhinosinusitis among the different immunodeficiency groups. There were no significant differences between chronic rhinosinusitis and conditions such as atopy, hypogammaglobulinemia, and treatments with immunoglobulin and/or azithromycin. The incidence of chronic rhinosinusitis was 77.8% (n = 7) in patients with a history of acute sinusitis and 20% (n = 7) in patients without a history of sinusitis, with a statistically significant difference between them (p = 0.002). Conclusion: Chronic rhinosinusitis is more common in patients with primary immunodeficiencies than in the normal population. For effective treatment, it is necessary to identify the factors that cause chronic rhinosinusitis. Further studies involving larger patient populations are needed to explain the mechanisms of chronic rhinosinusitis.

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Section: Methodsmentioning

confidence: 99%

Chronic Rhinosinusitis in Patients with Primary Immunodeficiency

Karadeniz

Aliyeva

Aydemir

et al. 2022

Int Arch Allergy Immunol

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“…Extensive flow cytometric analysis at diagnosis including T, B, natural killer (NK), T‐cell, and B‐cell subtypes was performed and compared between the two diseases (Figure 2A, Tables and ). Interestingly, in CTLA4 +/− patients, the normalized T‐cell compartment according to the age‐matched healthy controls reference values 21 demonstrated more skewing toward memory cells, characterized by diminished naive CD4 + T cells (CD45RA + CCR7 + ) and central memory CD4 + T cells (CD45RA − CCR7 + ) ( n = 11, 91.7% and n = 10, 83.3%) compared to LRBA −/− patients ( n = 12, 48.0% and n = 7, 28.0%) ( p = 0.040, p = 0.030, respectively). Additionally, effector memory CD4 + T cells (CD45RA − CCR7 − ) were significantly higher in CTLA4 +/− patients ( n = 11, 91.7%, p = 0.010).…”

Section: Resultsmentioning

confidence: 99%

“…Peripheral lymphocyte subset analyses and intracellular protein staining were performed by flow cytometry as described previously 13,18,20 and compared with age‐matched healthy reference values 21 . The FOXP3 and CTLA‐4 expressions were evaluated at diagnosis before starting abatacept treatment.…”

Section: Methodsmentioning

confidence: 99%

“…Peripheral lymphocyte subset analyses and intracellular protein staining were performed by flow cytometry as described previously 13,18,20 and compared with age-matched healthy reference values. 21 The FOXP3 and CTLA-4 expressions were evaluated at diagnosis before starting abatacept treatment. The ratio of mean fluorescence intensity (MFI) for intracellular CTLA-4 protein was determined by dividing that raw CTLA-4 MFI in memory Tregs (mTreg) cells by the raw CTLA-4 MFI of unstimulated CD4 + naive conventional T (nTcons, CD45RA + FOXP3 − ) cells, used as an internal control that does not express CTLA-4 protein.…”

Section: Flow Cytometric Analysismentioning

confidence: 99%

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Comparing the levels of CTLA‐4‐dependent biological defects in patients with LRBA deficiency and CTLA‐4 insufficiency

Catak

Akcam

Eltan

et al. 2022

Allergy

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Background Lipopolysaccharide‐responsive beige‐like anchor protein (LRBA) deficiency and cytotoxic T‐lymphocyte protein‐4 (CTLA‐4) insufficiency are recently described disorders that present with susceptibility to infections, autoimmunity, and lymphoproliferation. Clinical and immunological comparisons of the diseases with long‐term follow‐up have not been previously reported. We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting the genetic defect in patients with LRBA deficiency and CTLA‐4 insufficiency. Methods Patients were evaluated clinically with laboratory assessments for lymphocyte subsets, T follicular helper cells (TFH), LRBA expression, and expression of CD25, FOXP3, and CTLA4 in regulatory T cells (Tregs) at baseline and 16 h post‐stimulation. Results LRBA‐deficient patients (n = 29) showed significantly early age of symptom onset, higher rates of pneumonia, autoimmunity, chronic diarrhea, and failure to thrive compared to CTLA‐4 insufficiency (n = 12). In total, 29 patients received abatacept with favorable responses and the overall survival probability was not different between transplanted versus non‐transplanted patients in LRBA deficiency. Meanwhile, higher probability of survival was observed in CTLA‐4‐insufficient patients (p = 0.04). The T‐cell subsets showed more deviation to memory cells in CTLA‐4‐insufficiency, accompanied by low percentages of Treg and dysregulated cTFH cells response in both diseases. Cumulative numbers of autoimmunities positively correlated with cTFH frequencies. Baseline CTLA‐4 expression was significantly diminished in LRBA deficiency and CTLA‐4 insufficiency, but significant induction in CTLA‐4 was observed after short‐term T‐cell stimulation in LRBA deficiency and controls, while this elevation was less in CTLA‐4 insufficiency, allowing to differentiate this disease from LRBA deficiency with high sensitivity (87.5%) and specificity (90%). Conclusion This cohort provided detailed clinical and laboratory comparisons for LRBA deficiency and CTLA‐4 insufficiency. The flow cytometric approach is useful in predicting the defective gene; thus, targeted sequencing can be conducted to provide rapid diagnosis and treatment for these diseases impacting the CTLA‐4 pathway.

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“…Pediatric age‐matched reference values have been determined for the major lymphocyte populations according to Turkish pediatric populations 19 . Each value was determined as low/normal or high, according to reference values, and then compared with healthy controls.…”

Section: Methodsmentioning

confidence: 99%

Altered immune response in organic acidemia

Altun

Kıykım

Zübarioğlu

et al. 2022

Pediatrics International

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Background Most patients with organic acidemia suffer from recurrent infections. Although neutropenia has been reported in multiple studies, other components of the immune system have not been evaluated thoroughly. This study was conducted to assess the immune status of patients with organic acidemia (OA). Methods Thirty‐three patients with OA who were followed up in Istanbul University‐Cerrahpasa, Cerrahpasa School of Medicine, Nutrition and Metabolism Department and a total of 32 age‐ and sex‐matched healthy controls were enrolled to the study. The demographic and clinical data were recorded retrospectively from patient files. Complete blood counts, immunoglobulins, and lymphocyte immunophenotyping were recorded prospectively in a symptom‐ (infection‐) free period. Results Of the 33 patients enrolled to the study, 21 (88%) were diagnosed with methylmalonic acidemia, 10 (33%) with propionic acidemia, and two (6.6%) with isovaleric acidemia. The mean age of the patients with OA and healthy subjects were 5.89 ± 4.11 years and 5.34 ± 4.36, respectively (P = 0.602). Twenty‐nine (88%) of the patients had experienced frequent hospital admission, 13 (39%) were admitted to pediatric intensive care unit, and 18 (55%) suffered from sepsis. Naïve helper T cells and recent thymic emigrants were significantly lower in OAs (P < 0.001). Various defects in humoral immunity have also been documented including memory B cells and immunoglobulins. Conclusions Patients with OAs may show adaptive immune defects rendering them susceptible to infections. Metabolic reprogramming based on nutritional modifications may be a promising therapeutic option in the future.

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Reference values for T and B lymphocyte subpopulations in Turkish children and adults

Cited by 18 publications

References 14 publications

Chronic Rhinosinusitis in Patients with Primary Immunodeficiency

Chronic Rhinosinusitis in Patients with Primary Immunodeficiency

Comparing the levels of CTLA‐4‐dependent biological defects in patients with LRBA deficiency and CTLA‐4 insufficiency

Altered immune response in organic acidemia

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