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International journal of Immunopathology, allergology, infectology.

Comparative phenotypic analysis of T- and B-lymphocytes in patients with autoimmune lymphoproliferative syndrome and Wiskott-Aldrich syndrome

Sharapova S.O., Paschenko O.E., Guryanova I.E., Migas A.A., Kondratenko I.V., Belevtsev M.V.

1 Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk region, v. Borovliany, Belarus
2 Russian Children’s Clinical Hospital, Moscow, Russia

Objective: To identify phenotypic changes in the formation of T-and B -lymphocytes, regulatory cytokines (BAFF, IL10) in the peripheral blood of patients with autoimmune lymphoproliferative syndrome ( ALPS ) and Wiskott-Aldrich syndrome (WAS) as human model of inborn immunedysregulation.
Methods: Flowcytometric analysis of the following T- and B-cell subsets was performed in 23 patients, 12 patients with ALPS (8 boys, 4 girls and) and 11 boys with WAS, compared to age-matched controls: T cells (CD3 +), T -helper cells (CD3+CD4+), cytotoxic T cells (CD3+CD8+), natural killer cells (CD3-CD16+ CD56+), «thymic emigrants» (CD4+CD31+CD45RA+), regulatory T cells (CD4+CD25+CD127-), transitional (CD19+CD38++IgM++), CD21low, CD21lowCD38low, naïve (CD19+CD27-IgM+/IgD+), switched memory (CD19+CD27+IgM-/IgD-), marginal zone B cells (CD19+CD27+IgM+/IgD+), putative self-reactive B cells (CD19+IgD+IgMlow), putative regulatory B cells (CD19+CD24++CD38++), functionally immature B lymphocytes (CD19+CD21-, CD19+ CD21-CD38-, CD19+CD21-CD38++) and BAFF, IL10 serum levels were evaluated by ELISA assay.
Results and conclusions: Immunological manifestation of dysregulation by T -cell immunity is the reduction of recent thymic emigrants (CD4+CD45RA+CD31+) and regulatory T-lymphocytes (CD4+ CD25+CD127-) in patients with ALPS regardless the presence of mutation in the Fas gene and WAS associated with autoimmune pathology. Immunedysregulation appears relative expansion of B-lymphocytes with putative autoreactive properties (CD19+IgD+IgM-/low), functionally immature B lymphocytes (CD19+CD21-CD38-) in ALPS patients with genomic Fas mutation and WAS with related autoimmune diseases.


Autoimmune lymphoproliferative syndrome, Wiskott-Aldrich syndrome , thymic emigrant, regulatory T-lymphocytes, memory B-lymphocytes, functional immature B - lymphocytes, “autoreactive” B-lymphocytes.

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Sharapova S.O., Paschenko O.E., Guryanova I.E., Migas A.A., Kondratenko I.V., Belevtsev M.V. Immunopathology, allergology, infectology 2013; 4:67-80