Prevalence and outcome of intracranial haemorrhage in haemophiliacs – a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH)

Abstract

The frequency and outcome of ICH in haemophiliacs have not changed in our cohort over the past 20 years. Trauma at birth is an important risk factor for ICH in patients with haemophilia A or B. Intracranial haemorrhages in older children are rare, and a better outcome may be expected.

Clinical Characteristics and Prognostic Factors in Hemophiliacs with Intracranial Hemorrhage: A Single-Center, Retrospective Experience

“…The incidence of ICH has been found to range from 2.2 to 7.5 % in patients with hemophilia [5,6,[12][13][14]. In one study, the cumulative hazard of ICH for the entire cohort over the comprehensive follow-up period was 26.7 per 1000 patients, and the annual rate of ICH was 2.50 events per 1000 patients (95 % CI = 1.90-3.31) [11].…”

Haemophilia in the first years of life

“…in neonates and in the elderly [32]. ICH is a major cause of mortality, and morbidity is also considerable in surviving children, with possible neurological impairment and/or psycho-intellectual sequelae [34,35]. Even in countries where a dramatic improvement of life expectancy has been achieved for haemophilia patients, ICH remains as a major pediatric issue.…”

“…Nevertheless ICH sometimes occur and the significant incidence that has been observed in the first week of life in the case of laborious or instrumental delivery emphasizes the role of the traumatic obstetrical conditions [35]. In the latest series, in countries with a good standard of health care from North America and Europe, a rather uniform and steady incidence of 3.0-4.0% has been reported during the neonatal period [1,12,[33][34][35][36]. This represents a significantly higher risk by comparison with non-haemophilic newborns for whom ICH incidence was reported from 0.03% in low-risk delivery (elective caesarean) to 0.1% in high-risk delivery with vacuum extraction, suggesting that the major risk factor for haemorrhage is complicated labour [37].…”

Previously undiagnosed hemophilia patient with intracerebral hemorrhage

“…Medium form has 1%-4% and light form has 5%-25% factor concentrations respectively. [1][2][3][4] The most severe cases of hemophilia A has characteristic symptoms such as joint bleeding which shows itself when child first starts walking. Usually hemarthrosis and bleeding to other sites occurs in childhood and newborns.…”

“…ICH is still a major cause of mortality and morbidity in hemophilia patients. [1][2][3][4][5] Undiagnosed hemophilia associated with intracerebral hemorrhage 2 In hemophilic arthropathy, blood seeps into the joint space recurrently. This extravasation is followed by inflammation in synovia, destruction of cartilage tissue and bone, demonstrating the clinical picture of arthropathy.…”