Short-term intermittent prophylaxis post-intracranial hemorrhage in children with hemophilia

Novie Amelia Chozie, Fitri Primacakti, Made Citra Saraswati, Damayanti Sekarsari

Research output: Contribution to journalArticlepeer-review

Abstract

Background Intracranial hemorrhage (ICH) is one of the major bleeding events causing mortality and long-term morbidity in children with hemophilia, especially those who receive on-demand therapy. Objective To evaluate the outcome of children with hemophilia after ICH receiving short-term intermittent prophylactic treatment. Methods This retrospective study was conducted in the Department of Child Health, Dr. Cipto Mangunkusumo Hospital, Jakarta. Children ≤ 18 years of age with hemophilia presenting with ICH between 2015-2020 were included. We recorded patients’ demo-graphics, type and severity of hemophilia, the presence of factor VIII (FVIII) inhibitor, brain CT scan, treatment, and outcomes of these patients. Patients who received short-term intermittent prophylaxis using clotting factor concentrate (CFC) post-ICH episodes were observed for ICH recurrence. Results There were 19 episodes of ICH experienced by 18 patients, consisting of 16 patients with hemophilia A and 2 with hemophilia B. Patients’ median age was 4 years (range 0-16 years). Hemophilia was classified as severe in 13 patients, moderate in 4 patients, and mild in 1 patient. Thirteen episodes were preceded by head trauma. The most common clinical manifestation was seizures (13.2%). The most common type of ICH was subdural hematoma. Two patients died and 2 patients had neurological sequelae dur-ing hospitalization. The median dose of short-term intermittent prophylaxis using CFC (n=16) was 20 IU/kg of FVIII twice a week and 30 IU/kg of FIX twice a week, for a median duration of 8 weeks (range 5-12 weeks). One patient who did not adhere to the prophylaxis regimen had recurrent ICH at a similar location 6 months after the first episode. Conclusion Our findings suggest that short-term intermittent prophylaxis is important to prevent the recurrence of ICH in children with hemophilia.

Original languageEnglish
Pages (from-to)173-178
Number of pages6
JournalPaediatrica Indonesiana(Paediatrica Indonesiana)
Volume62
Issue number3
DOIs
Publication statusPublished - May 2022

Keywords

  • hemophilia
  • intracranial hemorrhage
  • prophylaxis

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