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Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Brown KL, Wekell P, Osla V, Sundqvist M, Sävman K, Fasth A, Karlsson A, Berg S - BMC Pediatr (2010)

Bottom Line: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval.IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Rheumatology and Inflammation Research, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. kelly.brown@gu.se

ABSTRACT

Background: This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.

Methods: A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.

Results: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.

Conclusions: Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.

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Related in: MedlinePlus

Scatter plot of white blood cells. The abundance (x 109/L) of white blood cells (WBC; top left), lymphocytes (top centre), monocytes (top right), neutrophils (bottom left), eosinophils (bottom centre) and basophils (bottom right). Absolute cell numbers are reported in Additional File 3, Table S2.
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Figure 2: Scatter plot of white blood cells. The abundance (x 109/L) of white blood cells (WBC; top left), lymphocytes (top centre), monocytes (top right), neutrophils (bottom left), eosinophils (bottom centre) and basophils (bottom right). Absolute cell numbers are reported in Additional File 3, Table S2.

Mentions: The concentration of RBC, thrombocytes, WBC and constituent WBC subtypes (monocytes, lymphocytes, neutrophils, basophils and eosinophils) were evaluated in blood drawn from controls and patients. Data are presented in Figure 1, Figure 2 and Additional File 3, Table S2. The concentrations of both RBC and WBC in AFP samples were comparable to that in controls. The absolute numbers of thrombocytes in AFP blood however exceeded the upper limit of the normal range and were 1.5 ± 0.1 fold higher than thrombocytes in blood from FP and controls.


Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Brown KL, Wekell P, Osla V, Sundqvist M, Sävman K, Fasth A, Karlsson A, Berg S - BMC Pediatr (2010)

Scatter plot of white blood cells. The abundance (x 109/L) of white blood cells (WBC; top left), lymphocytes (top centre), monocytes (top right), neutrophils (bottom left), eosinophils (bottom centre) and basophils (bottom right). Absolute cell numbers are reported in Additional File 3, Table S2.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2944328&req=5

Figure 2: Scatter plot of white blood cells. The abundance (x 109/L) of white blood cells (WBC; top left), lymphocytes (top centre), monocytes (top right), neutrophils (bottom left), eosinophils (bottom centre) and basophils (bottom right). Absolute cell numbers are reported in Additional File 3, Table S2.
Mentions: The concentration of RBC, thrombocytes, WBC and constituent WBC subtypes (monocytes, lymphocytes, neutrophils, basophils and eosinophils) were evaluated in blood drawn from controls and patients. Data are presented in Figure 1, Figure 2 and Additional File 3, Table S2. The concentrations of both RBC and WBC in AFP samples were comparable to that in controls. The absolute numbers of thrombocytes in AFP blood however exceeded the upper limit of the normal range and were 1.5 ± 0.1 fold higher than thrombocytes in blood from FP and controls.

Bottom Line: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval.IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Rheumatology and Inflammation Research, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. kelly.brown@gu.se

ABSTRACT

Background: This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.

Methods: A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.

Results: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.

Conclusions: Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.

Show MeSH
Related in: MedlinePlus