Clinical and immunological characteristics of primary immunodeficiencies with chronic non-malignant lymphoproliferation

Zharankova Yu.S., Aleshkevich S.N., Sharapova S.O., Sakovich I.S., Guryanova I.E., Polyakova E.A., Shitikova M.G., Uglova T.A., Belevtsev M.V.

Republican Scientific and Practical Center for Pediatric Oncology, Hematology and Immunology, Minsk, Belarus

Aim. To increase the efficiency of diagnosis of primary immunodeficiencies occurring with chronic non-malignant lymphoproliferation through the use of significant clinical and immunological biomarkers.
Materials and methods. Clinical, immunological, immunohistochemical and molecular genetic research methods were used in the work. The study included patients of the Republic of Belarus with an established diagnosis of PID (n=58; 13.1% of the total number of patients with PID), whose onset of the disease included lymphadenopathy, splenomegaly and/or extranodal lymphoproliferative lesions, which we discovered during a clinical study of patients (through physical examination, computed tomography, magnetic resonance imaging and ultrasound). The immunological study was carried out using multicolor immunophenotyping on a Navios flow cytofluorimeter (Beckman Coulter) using no-wash sample preparation technology. A set of independent diagnostic criteria for PID-associated chronic non-malignant lymphoproliferation was determined using a multivariate binary logistic regression method.
Conclusion. Primary immunodeficiencies with chronic non-malignant lymphoproliferation are characterized by clinical and immunological variability. For the first time in the Republic of Belarus, the spectrum of immunological changes in patients with PID-associated chronic non-malignant lymphoproliferation was analyzed. The most informative diagnostic criteria from among the standard and minor populations of lymphocytes are double-negative T-lymphocytes, CD19+ and CD3+HLA-DR+.