B. Faganel Kotnik
Klinični oddelek za otroško hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana

J. Jazbec
Klinični oddelek za hematologijo in onkologijo, Pediatrična klinika Ljubljana, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

L. Kitanovski
Klinični oddelek za hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana

Abstract

Primary immune thrombocytopenia (ITP) is an acquired immune-mediated disorder characterised by isolated thrombocytopenia in the absence of any obvious initiating and/or underlying cause of the thrombocytopenia. Children with ITP are at risk of intracranial bleeding and bleeding in the chest or abdomen. The diagnosis is based on a typical clinical history and examination. In newly diagnosed ITP, the platelet count usually normalises in two to four months, hence treatment is reserved only for patients at increased risk of serious bleeding. Newly diagnosed ITP is treated with intravenous immunoglobulins (IVIg) and glucocorticoids, less often with anti-Rh-D immunoglobulins and only exceptionally with splenectomy. In persistent or chronic ITP, children usually do not bleed significantly. In half of the children with chronic ITP, the platelet count stabilises over time. Only exceptionally is treatment with medications or splenectomy necessary. Chronic ITP is more resistant to treatment than newly diagnosed ITP. After splenectomy, remission is achieved in 60 % of children. Other treatment options for chronic ITP are intravenous immunoglobulins, glucocorticoids, anti-Rh-D immunoglobulins, rituximab, immunosuppressives, vincristine, Danazol, growth factors for platelets (romiplostim and eltrombopag) and antimicrobial treatment of infection with Helicobacter pylori, when proven.

Key words: thrombocytopenia, immune system, bleeding, immunoglobulins, splenectomy, glucocorticoids, immunosupressives, Helicobacter pylori.