SEVERE HAEMOPHILIA A: CASE REPORT

Abstract

Background: Haemophilia A is the most common inherited coagulation disorder (~1 in 5,000 male births) caused by factor VIII (FVIII) deficiency. Severe disease (FVIII <1%) presents in infancy with spontaneous or trauma-related bleeding. While prophylactic FVIII therapy has improved outcomes in high-income countries, many patients in low- and middle-income settings receive only on-demand treatment. Case summary: A male child presented at around one year of age with persistent bleeding after head trauma. Laboratory evaluation showed prolonged APTT, and a correction study confirmed FVIII deficiency. Factor assay demonstrated FVIII activity <1%, establishing severe Haemophilia A. Over the next eighteen years, recurrent bleeding episodes were managed exclusively with on-demand plasma transfusions without FVIII prophylaxis. Outcome: The patient survived repeated bleeding episodes but probable cumulative joint damage remains a concern. No inhibitor screening was documented. Severe Haemophilia A may present without family history. On-demand plasma therapy does not prevent arthropathy, both prophylaxis and inhibitor surveillance are essential.