Intracranial Hemorrhage in Egyptian Children with Primary Immune Thrombocytopenia (ITP): Report of 24 Cases in 20 Years

Abstract

Background: 35 million children < 18 years; approximately 1800 new cases of ITP were diagnosed annually in Egypt. Intracranial hemorrhage (ICH) is a rare devastating complication of childhood immune thrombocytopenia (ITP). Incidence of ICH among children with ITP varies markedly in different studies from 0.2 up to 1.0 %. Intracranial hemorrhage after head trauma in children with ITP leads to significant morbidity and mortality. We published data during 1997 - 2007 (10 ICH) in children with primary ITP; risk factors and outcome. Aim & Methodology: A follow-up study to assess any change in outcome of ICH from last decade; whether new therapies might change the landscape of ICH. Centers treating > 25 child with prim ITP /year and offered a complete data of >150 children with ITP over 2 decades were enrolled. We compared 2008 - 2018 with the decade before it; variation of ICH reporting from center to center and outcome in relation to therapy. All children with ITP and ICH during study period had been treated, within < 24 hours and referred to neurosurgical hospital complex facility for consultation and intervention. Time elapsed till receiving platelet enhancing therapy and neurosurgical intervention was assessed, Outcome whether a complete recovery, permanent sequalae or death was reported. Results: Four thousand, three-hundred and forty primary ITP were evaluated, ( 380 were excluded due to incomplete data ) Twenty-four (0.6%) ICH were reported over 2 decades (14 in this decade with 20% increase incidence) and 48 matched ITP control subjects were evaluated. Platelet counts were less than 10 x 10(9)/L in 90% of children with ICH. Four (16.2%) children developed ICH within 14 days of diagnosis of ITP; one of these, was the presenting feature of ITP. four were from 3-12 months and sixteen (66.6%) of children had chronic ITP. Centers treating > 50 case/year had a higher frequency of reporting 0.8 % compared to 0.2 % in centers < 50 cases/year. Outcome is better on early intervention as well as aggressive platelet enhancing therapy with 70% complete recovery compared with 30% complete recovery on delayed intervention. Head trauma and hematuria and PC < 10 were the mostly associated with ICH, identified in 33%, 25% and 90% respectively of the patients with ICH and in 1, none and 50% of the controls (P < .001). Bleeding beyond petechiae and ecchymoses was also linked to ICH. Mortality was 25%; a further 25% had neurologic sequelae. Neurosurgical intervention was done in 25% of cases with good outcome. Reporting was more in this decade with better outcome in bigger centers. Conclusion: A rise in the incidence of ICH in Children with severe thrombocytopenia over last decade; high risk for ICH among those with PC< 10,000 plus head trauma and/or hematuria. Platelet enhancing agents whether HDMP or IVIG or TPO-RAs could not prevent ICH. However they had a good impact on survival and lessen sequalae if used in combination. Strategies by which high-risk children could be identified and well managed in small centers. Disclosures No relevant conflicts of interest to declare.

New concepts in pathogenesis of primary immune thrombocytopenia

“…After 60 years, men reclaim their dominance. In Egypt, over 1800 new cases of ITP are detected each year (3) .…”