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Complex immunophenotyping single-cell analysis are essential for systems biology and cytomics.
The application of cytomics in immunology and cardiac research and diagnostics is very broad,
ranging from the better understanding of the cardiovascular cell biology to the identification of heart
function and immune consequences after surgery. TCPC or Fontan-type circulation is an accepted
palliative surgery for patients with a functionally univentricular heart. Protein-losing enteropathy
(PLE), the enteric loss of proteins, is a potential late complication after TCPC surgery. PLE etiology
is poorly understood, but immunological factors seem to play a role. This study was aimed to gain
insight into immune phenotype alterations following post-TCPC PLE. Patients were studied during
routine follow-up up to 5yrs after surgery, blood samples of TCPC patients without (n=21, age
6.8±2.6 years at surgery; mean±SD) and with manifest PLE (n=12, age 12.8± 4.5 years at sampling)
and age matched healthy children (control, n=22, age 8.6±2.5 years) were collected. Routine
laboratory, immune phenotype and serological parameters were determined. Following PLE the
immune phenotype dramatically changed with signs of acute inflammation (increased neutrophil and
monocyte count, CRP, IL-8). In contrast, lymphocyte count (NK-cells, αβTCR+CD4+, αβTCR+CD8+ cells) decreased (p<0.001). The residual T-cells had elevated CD25 and CD69
expression. In PLE-patients unique cell populations with CD3+αβ/γδTCR- and αβTCR+CD4-8-
phenotype were present in increased frequencies. Our studies show dramatically altered leukocyte
phenotype after PLE in TCPC patients. These alterations resemble to changes in autoimmune
diseases. We conclude that autoimmune processes may play a role in etiology and pathophysiology
of PLE.
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