Naslov
Neonatal Hemophagocytic Lymphohistiocytosis - Case Report

Autori
Roganović, Jelena ; Kvenić, Barbara ; Jonjić, Nives ; Seili-Bekafigo Irena ; Kardum-Skelin, Ika

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

Izvornik
Knjiga sažetaka 4. Hrvatski kongres kliničke citologije, 1. Hrvatski simpozij analitičke citologije i 2. Hrvatski simpozij citotehnologije s međunarodnim sudjelovanjem / Kardum-Skelin, Ika ; Batinić, Drago ; Anić, Veronika - Zagreb : Hrvatsko društvo za kliničku citologiju ; Hrvatska udruga citotehnologa, 2009

Skup
4. Hrvatski kongres kliničke citologije, 1. Hrvatski simpozij analitičke citologije i 2. Hrvatski simpozij citotehnologije s međunarodnim sudjelovanjem

Mjesto i datum
Split, Hrvatska, 11.10.2009. - 14.10.2009

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Hemophagocytic lymphohystiocytosis

Sažetak
Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature is an accumulation of activated T lymphocytes and macrophages predominantly in lymphoid tissues. Although it can occur in all age groups, neonatal-onset HLH is very rare. We report on a case of HLH presenting with anemia and respiratory distress at birth. Several weeks prior to diagnosis the symptoms were attributed to a systemic infection. The child developed typical clinical and laboratory findings, and was diagnosed with HLH according to the HLH-2004 guidelines. Chemo-immunotherapy was initiated, but after a temporary control of the disease he succumbed to rapidly progressive HLH. Post-mortem, extensive hemophagocytosis was found in multiple organs. No specific genetic defect was identified. HLH is potentially fatal childhood disease. It is important for pediatricians to be able to early identify

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti

POVEZANOST RADA