BackgroundKlippel–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 presentationWe 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.ConclusionsThis 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.

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.

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