ABSTRACT
Age and gender-related variability of main lymphocyte subsets (T, B and NK cell absolute counts and percentages from Ly; T4, T8 and DN cell absolute counts and percentages from lymphocytes and from T cells; T4:T8 and T:B ratios) was studied in a large cohort of pediatric patients (2 days - 17 years) at yearly intervals. 4128 6-color TBNK tests performed on BD FACSCanto II flow cytometer were assessed; patients with immune deficiencies and tumors were not included. The study revealed significant age and gender-related changes in all subsets. Absolute counts of T, B, T4 cells dropped from neonates to adolescents, decrease of T8 and NK cells was milder; relative count of T cells increased with age and that of B cells decreased; T4:T8 ratio went down and T:B ratio grew. Total T, T4 cells and T4:T8 ratio were significantly higher in girls, while T8, NK and DN cells - in boys; significantly higher relative and absolute B cell counts in boys appeared in adolescence. We compared our results with reference values for healthy children (Tosato et al., 2015), there was a good concordance, except for DN cells. Advantages of using patient cohort instead of healthy children as reference, possibilities for adjusting age and gender-specific reference ranges and potential international data pooling are discussed. This article is protected by copyright. All rights reserved.
ABSTRACT
BACKGROUND: Klippel-Trenaunay-Weber syndrome is a rare syndrome; unfortunately, very few studies of the connection between hypersplenism, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome have been published. CASE PRESENTATION: We report the case of a 40-year-old white man with a typical clinical presentation of Klippel-Trenaunay-Weber syndrome, including "port-wine stains," varicose veins, hypertrophy of lower extremities, and arteriovenous fistula, as well as an unfortunate development of hypersplenism and nephrotic syndrome. CONCLUSIONS: This case report described considerable atypical relevance of Klippel-Trenaunay-Weber syndrome and hypersplenism together with nephrotic syndrome. A multidisciplinary approach was made. Unfortunately, hypersplenism is characterized by pancytopenia that suggests splenectomy, whereas nephrotic syndrome is an indication for renal biopsy; the splenectomy and renal biopsy were delayed due to our patient's severe condition. Deeper analysis including study of other patients with Klippel-Trenaunay-Weber syndrome would help us to understand the connection between elevated spleen and liver sizes, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome.
