HEMOPHILIA-A - 2 CASES SHOWING UNUSUAL FEATURES AT BIRTH

We report two patients with severe haemophilia A, factor VIII < 2 u dL (-1), diagnosed at birth, through isolated bleeding in the adrenal gla nd (case 1) and through a haematoma in the liver (case 2). In these tw o clinical cases, the vital emergency, with haemorrhagic shock, requir ed early diagnosis of the hereditary coagulation defect. Generally at birth, any unexplained bleeding should prompt screening for haemophili a. In the newborn period, the PTT is inadequate for many reasons and t he laboratory evaluation must include factor VIII and IX levels.