Haemophilia : the official journal of the World Federation of Hemophilia · 2026
Central Nervous System Bleeding in Children With Haemophilia in Limited Resource.
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Komvilaisak Patcharee, Sirichaipornsak Sudarat, Sripraya Paiporn, Laoaroon Napat, Suwannaying Kunanya, Duanthongphon Pichayen
Аннотация
INTRODUCTION: Central nervous system (CNS) bleeding in children with haemophilia is a life-threatening complication that may cause severe neurological sequelae or death. In resource-limited settings, where prophylaxis is not universally accessible, its patterns and outcomes may differ from those in high-income countries.
AIMS: To describe the clinical characteristics, management and outcomes of paediatric haemophilia patients with CNS bleeding.
METHODS: This retrospective descriptive study reviewed 19 children with haemophilia who developed CNS bleeding.
RESULTS: The median age at onset was 1.8 years (range, 0.4-14.3). Seventeen patients had Haemophilia A (11 severe, 4 moderate, 2 mild), and two had Haemophilia B (1 severe, 1 moderate). Three Haemophilia A patients developed high-titre inhibitors with recurrent intracerebral haemorrhage (ICH). Bleeding types included ICH (13), subdural haemorrhage (8), subarachnoid haemorrhage (3), intraventricular haemorrhage (1), epidural haematoma (1), subgaleal haematoma (1) and spinal epidural haematoma (1). Most events were spontaneous (17), while trauma (5) and lumbar puncture (2) were reported precipitating causes. Presentations included seizures (12), altered consciousness (13), headache/vomiting (8) and back pain (1). Four children were newly diagnosed with Haemophilia A at the time of CNS bleeding. Six required craniotomy, while others without inhibitors were successfully managed with factor replacement. Outcomes included death (4), developmental delay (5), hemiparesis (3) and full recovery (10). Deaths were mainly associated with delayed recognition and treatment.
CONCLUSION: Early recognition of CNS bleeding is essential for timely factor replacement and improved survival. Key strategies include caregiver education, healthcare provider training, streamlined emergency pathways and wider access to prophylaxis.
AIMS: To describe the clinical characteristics, management and outcomes of paediatric haemophilia patients with CNS bleeding.
METHODS: This retrospective descriptive study reviewed 19 children with haemophilia who developed CNS bleeding.
RESULTS: The median age at onset was 1.8 years (range, 0.4-14.3). Seventeen patients had Haemophilia A (11 severe, 4 moderate, 2 mild), and two had Haemophilia B (1 severe, 1 moderate). Three Haemophilia A patients developed high-titre inhibitors with recurrent intracerebral haemorrhage (ICH). Bleeding types included ICH (13), subdural haemorrhage (8), subarachnoid haemorrhage (3), intraventricular haemorrhage (1), epidural haematoma (1), subgaleal haematoma (1) and spinal epidural haematoma (1). Most events were spontaneous (17), while trauma (5) and lumbar puncture (2) were reported precipitating causes. Presentations included seizures (12), altered consciousness (13), headache/vomiting (8) and back pain (1). Four children were newly diagnosed with Haemophilia A at the time of CNS bleeding. Six required craniotomy, while others without inhibitors were successfully managed with factor replacement. Outcomes included death (4), developmental delay (5), hemiparesis (3) and full recovery (10). Deaths were mainly associated with delayed recognition and treatment.
CONCLUSION: Early recognition of CNS bleeding is essential for timely factor replacement and improved survival. Key strategies include caregiver education, healthcare provider training, streamlined emergency pathways and wider access to prophylaxis.