Intracranial haemorrhage among a population of haemophilic patients in Brazil

Abstract

Intracranial haemorrhage (ICH) is a common cause of morbidity and mortality in haemophilic patients. The overall incidence of ICH has been reported to range from 2.2% to 7.5% in patients with haemophilia. From 1987 to 2001, 401 haemophilic patients from the Serviço de Hemofilia, Disciplina de Hematologia e Hemoterapia, Universidade Federal de São Paulo were evaluated. The episodes of ICH were documented by CT scan and the anatomic location, clinical presentation, relationship to trauma and clinical factors, including the presence of HIV infection and the presence of inhibitor, were reviewed. Among 401 haemophilic patients, 45 ICH episodes in 35 (8.7%) patients with age ranging from 4 days to 49 years (mean 10.6 years) were observed. A history of recent trauma was documented in 24 (53.3%) cases. Seventeen (37.8%) episodes occurred in more than one site of bleeding, 12 (26.7%) were subdural, seven (15.5%) subarachnoid, four (8.9%) epidural, two (4.4%) intracerebral and one (2.2%) intraventricular. The most frequent symptoms were headache and drowsiness. All patients were submitted to replacement therapy and neurosurgical intervention was performed in eight (17.8%) patients. Despite the treatment, three (8.6%) haemophilia A patients died due to the ICH event and three presented late sequelae. The most important aspect of ICH management is the early replacement therapy in haemophilic patients. This prompt treatment will increase the chances of a better prognosis. Another impact measure consists in the administration of the deficient coagulation factor after every head trauma, even when considered minor.

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

“…In the present series, 3 (37.5 %, 3/8) developed neurological complications, including transient neurological abnormalities (blurred vision and dysarthria) and two patients achieved complete recovery from symptoms. Previous studies have shown variability in the incidence of long-term sequelae, ranging from 9.4 to 22.7 % [5,6,11,16]. This is also consistent with our results.…”

“…The mortality rate was 20.0 % (2/10) in our clinical series of patients. This is consistent with previous reports showing variability in mortality ranging from 0 to 25 % in patients with hemophilia presenting with ICH [5,6,11,16]. Two of our three patients who underwent neurosurgical intervention died, but this was likely the result of the ICH characteristics and patient conditions, not the neurosurgical intervention.…”

“…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]. Patients with hemophilia are 20 to 50 times more likely to develop ICH than the general population, with a reported prevalence of 2.7-12 % and an incidence of 290-748/10 5 patient-years [3][4][5][6].…”

“…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].…”

“…Intracranial hemorrhage (ICH) is the most serious bleeding event that occurs in patients with hemophilia; its estimated mortality rate is approximately 20 %, accounting for the largest number of deaths from bleeding [1,[3][4][5]. ICH may occur spontaneously or due to trauma (spontaneous or traumatic ICH, respectively).…”