Associations between intracranial haemorrhage and prescribed prophylaxis in a large cohort of haemophilia patients in the United States

Abstract

Summary Intracranial haemorrhage (ICH) is the most serious type of bleeding for patients with haemophilia. Prior published reports regarding ICH predate the widespread provision of prophylaxis. Our study objectives were to determine risk factors for ICH and whether prophylaxis reduces ICH occurrence. We performed a nested case‐control study of persons with haemophilia, ≥2 years of age enrolled in the Centers for Disease Control and Prevention Universal Data Collection project. Of 10 262 patients 199 (1·9%) experienced an ICH for an incidence rate of 390/105 patient years. Head trauma was reported in 44% (88/199). ICH mortality was 19·6% (39/199). Significant risk factors for ICH included a high titre inhibitor [odds ratio (OR) = 4·01, 95% confidence interval (2·40–6·71)], prior ICH [OR = 3·62 (2·66–4·92)] and severe haemophilia [OR = 3·25 (2·01–5·25)]. Prophylaxis was associated with a significant risk reduction for ICH occurrence in patients with severe haemophilia who were negative for human immunodeficiency virus or an inhibitor, with an OR of 0·52 (0·34–0·81) and 0·50 (0·32–0·77) respectively. The most significant risk factors for ICH included the presence of an inhibitor, prior ICH, severity of haemophilia and reported head trauma. This is the first study to demonstrate that prescribed prophylaxis conferred a protective effect against ICH in patients with uncomplicated severe disease.

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

“…This treatment is reportedly effective in preventing joint hemarthrosis [21]. One cohort study examined whether prophylaxis is effective in lowering the risk of ICH and found that it was effective in significantly lowering the risk of ICH in HIV-negative patients with severe hemophilia or those with inhibitors [7]. In that study, the mortality was 19.6 % and its burden was shifted to adults.…”

“…Traumatic ICH is more prevalent in children than adults, and head trauma, especially, is one of the most common injuries in childhood [3,6]. Predisposing risk factors for ICH include the severity of the hemophilia, advanced age, the presence of inhibitors, human immunodeficiency virus (HIV) infection, hypertension, and a history of ICH [1,6,7].…”

Reasons for admission to emergency services of the patients with hemophilia

“…Travma yakınması ile başvuran hastaların fizik muayenesi dikkatlice yapılmalıdır. Radyasyon etkisi nedeniyle hastaların fizik muayene bulgularına göre bilgisayarlı tomografi görüntülemesi önerilmektedir [8,9]. Biz de kafa travması olan olgulara erken dönemde faktör tedavisi vermemiz nedeniyle kanama bulgusu olmadığını düşünmekteyiz.…”

Intracranial haemorrhage in a children suffering from heamofilia

“…Необходимо отметить, что в ис-следованиях общенациональных когорт пациентов с гемофилией детей в возрасте от 0 дней до 15-20 лет было от 47,2 до 100 % (табл. 1) [3][4][5][6][7][8].…”

“…2) [3,4,[6][7][8]. Однако в Германии в 1999 г. был проведен анализ 27 де-тей с ВЧК, больных гемофилией, в возрасте от 0 до 3 лет.…”