Changes in the occurrence of and risk factors for hemophilia‐associated intracranial hemorrhage

Abstract

Recent studies suggest that rates of intracranial hemorrhage (ICH) increased concomitant with the HIV epidemic among the hemophilia population, but no studies have directly examined factors associated with ICH. To determine ICH rates and identify factors associated with ICH, we performed a nested case-control study of a cohort composed of all hemophilic males identified by a surveillance system. Data were obtained from medical records of care received during 1993-1997. Patients with ICH listed in hospital records or on death certificates during the 5-year period were compared to the remainder of the cohort to examine associations between ICH and patients' demographic and clinical factors including the presence of HIV infection. Among the 3,269 males in the cohort, 88 (2.7%) had an ICH during follow-up, an average incidence rate of 0.0054 case/year. Hemorrhage sites were intracerebral for 37.5%, subdural for 34.1%, unspecified for 19.3%, subarachnoid for 12.5%, and epidural for 8% of cases. For 22% of cases, the ICH was trauma-related, and, overall, 16 patients (18.2%) died. Several factors were independently associated with ICH (odds ratio, P value): severe disease (2.0, 0.05); age 51+ years compared to 6-10 year olds (3.7, 0.02); presence of an inhibitor (3.5, <0.001); and HIV infection among whites only (4.0, <0.001). ICH rates in our cohort were 2-fold higher compared to rates from previous reports. Much of the increase was attributed to HIV infection, which raised ICH risk primarily in whites and was frequently associated with spontaneous ICH among older individuals. Am.

A Review on Hemophilia in Children

“…Most common first bleeding sites are shown table 15. Similar pattern of bleeding is also reported by another large hospital based retrospective study in 399 newborn 16,17 .Intracranial hemorrhage (ICH), though rare, is the serious complication often misdiagnosed in neonatal period because of vague presentation especially with negative family history. High index of suspicions are necessary to pick these cases.…”

“…Intracranial hemorrhage: Intracranial hemorrhage is the serious complication of hemophilia with a significant cause of disability and long term neurological sequelae as many as 60-75% cases 16,17 . Prevalence of symptomatic ICH in all ages varies from 3-12% with a substantial proportion occurring in children 26 .…”

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

“…Several reports have suggested an increased incidence of clinically significant intracranial hemorrhage and worse outcomes for patients with hemophilia and inhibitors. [24][25][26][27][28][29] Our study was not powered to detect the benefit of prophylaxis for low-incidence, high-morbidity bleeding events such as intracranial hemorrhage. However, it is reasonable to assume that patients who have a good response to prophylaxis are also likely to have a reduced risk of life-threatening bleeding.…”

The Aging Patient with Hemophilia: Complications, Comorbidities, and Management Issues

“…5,14,23 Data from the six-state Hemophilia Surveillance System Project cohort in the United States demonstrated that age greater than 51 years, severe hemophilia, HIV, and the presence of inhibitor were independent risk factors for intracranial hemorrhage. 24 An effect of inhibitor presence on intracranial hemorrhage-associated mortality, however, was not confirmed by UK hemophilia surveillance data. 25 Intracranial hemorrhage has the highest mortality of any hemophilia-related bleeding, and a death rate of 30% has been reported among hemophilia patients age 50 and older with intracranial hemorrhage.…”