BLASTIC PLAZMATSITOIDS DENDRITOCELL TUMOUR: EXPERIENCE OF DIAGNOSTICS AND TREATMENT IN THE SVERDLOVSK REGIONAL ONCOHEMATOLOGICAL CENTER

Aim: to analyze the case of blastic plasmacytoid dendritic cell neoplasm (BPDN) detection and treatment in Sverdlovsk Regional Hematological Centre (Ekaterinburg). Bone marrow and peripheral blood samples obtained from female, aged 32, treated in Sverdlovsk Regional Hematological Centre during the Jan-Marth 2014. Diagnosis of BPDN verified using cytology, cytochemistry and immunophenotyping. Cytogenetic analysis was realized using G-banding. Detection of mutations in exons 12-13 ASXL1, exons 18-26 DNMT3A, exons 12-15 and 19-21 FLT3, exons 7-12 and 16-19 KIT, exons 1-4 KRAS, exons 9-12 NPM1, exons 1-4 NRAS, exons 4-11 ТР53, exons 6-9 WT1 genes was performed using direct sequencing. Cytological characterization of BPDN blast cells: large cells with oval, reniform nuclei with delicate chromatin structure, with one or two, at least - several small nucleoli, with wide basophilic cytoplasm and bright perinuclear area, richly vacuolated. In cytochemistry lipids is positive in 10.0 % of blasts, glycogen -6.0 %. The primary method of differential diagnosis of BPDN in case study was the identification of blast cells in the bone marrow. Immunophenotype of blast cells was CD4, CD15, CD33, CD38, CD56, CD64, CD65, HLA-DR(+), MPO-cyt (+/-). Karyotype was 48, XX, add(1)(p36), +6, +8 [8]. In case 2 gene mutations were co-existed in samples: KIT gene insertion and TP53 gene non-synonymous substitution с. 215 C>G. Coding sequence the remaining exons of the investigated genes is fully consistent with "wild type".

иммунофенотип, бластная плазмоцитоидная дендритоклеточная опухоль, кариотип, прямое автоматическое секвенирование, immunophenotyping, blastic plasmacytoid dendritic cell neoplasm, cytogenetics, direct sequencing