Naslov
Case study report : Classical ataxia- telangiectasia patient with severe B cell immunodeficiency and expansion of CD3+HLA-DR+ T cells

Autori
Bulat Lokas, Sandra ; Živković, Jelena ; Banić, Ivana ; Navratil, Marta ; Mrkić Koba , Iva ; Polančec, Denis ; Turkalj, Mirjana

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
30th Congress of the International Society for Advancement of Cytometry : abstracts / - , 2015, Xx-xx

Skup
30th Congress of the International Society for Advancement of Cytometry (30 ; 2015)

Mjesto i datum
Glasgow, Ujedinjeno Kraljevstvo, 26.06.2015. - 30.06.2015

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Ataxia-telangiectasia, B cell, flow cytometry
(Ataxia-telangiectasia, , B cell, flow cytometry)

Sažetak
We present the case of an 15-yeard old boy clinically monitored due to ataxia- telangiectasia (A-T). The patient has been monthly administred at our hospital for intravenous immunoglobulin (IVIG) substitution (400 mg/kg every month), anti-asthma therapy, respiratory physical therapy and aggressive antibiotic treatment. Lately, his immunologic status assessment before treatment involves comprehensive immunophenotypization by flow cytometry. Comprehensive immunophenotyping of whole blood was performed using staining for cell surface markers CD45, CD3, CD4, CD8, CD19, CD56, CD16, HLA-DR and CD27 and lyse &fix / wash protocol. Acquisition of samples was performed using the Navios flow cytometer (Beckman Coulter, USA). Data was analyzed using the FlowLogic software package (Inivai Technologies, Australia). Severe B cell deficiency was detected in peripheral blood from A-T patient. Patient had low relative and absolute number of total B cells and lower absolute number of memory B cells (CD19+CD27+), while relative number of memory B cells was decreased. In addition, threefold increase in relative and absolute number of activated CD3+HLA-DR+ T cells was detected. The disturbance of B cell and T cell homeostasis at one A-T patient was detected by polychromatic flow cytometry. Hypogammaglobulinemia accompained with combined T- cell and B-cell disorder will be further monitored with additional panels for the detection of T cells and B cells subsets.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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