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Diagnosis, factor replacement and bleeding prevention
Haemophilia A and B are X-linked deficiencies of factor VIII and IX; von Willebrand disease is the most common inherited bleeding disorder. Diagnosis requires factor assays and careful history. Management includes factor replacement, antifibrinolytics, desmopressin in selected cases, and preventive planning for surgery or pregnancy.
Haematology & Haemato-Oncology
Authored and reviewed by Dr. Sachita Baniya (MBBS, FCPS (Clinical Haematology), NMC 20158). Evidence-based, no fabricated outcomes.
Haemophilia A and B are X-linked deficiencies of factor VIII and IX; von Willebrand disease is the most common inherited bleeding disorder. Diagnosis requires factor assays and careful history. Management includes factor replacement, antifibrinolytics, desmopressin in selected cases, and preventive planning for surgery or pregnancy.
Reviewed by Dr. Sachita Baniya, MBBS, FCPS — Consultant Haemato-Oncologist & Clinical Hematologist (NMC 20158). For evaluation, contact the respective hospital or cancer centre — Kathmandu Cancer Center (KCC) Nepal, Civil Service Hospital Kathmandu, or Advanced Care Hospital.
A bleeding history — prolonged bleeding after surgery, dental extraction, childbirth or trauma, and family history — prompts coagulation testing. Factor VIII, IX and von Willebrand factor assays confirm the diagnosis and severity. Platelet function testing may be needed for von Willebrand disease subtypes.
Haemophilia treatment uses factor replacement, with prophylaxis in severe disease to prevent joint damage. Desmopressin is useful in mild haemophilia A and some von Willebrand disease. Antifibrinolytics help with mucosal bleeding. Pregnancy, surgery and dental procedures require coordinated planning with a haematology team.
Guidelines referenced:
World Federation of Hemophilia treatment guidelinesBritish Society for Haematology haemophilia guidelinesISTH guidance on von Willebrand diseaseContent on this website is for general educational purposes only and does not constitute medical advice, diagnosis, or treatment. It is not a substitute for evaluation by a qualified healthcare professional. Always seek the advice of your physician or other qualified provider with any questions regarding a medical condition. Never disregard professional medical advice or delay seeking it because of something you have read on this website. In case of a medical emergency, contact emergency services immediately. No doctor–patient relationship is established by use of this site or by contacting the clinic through the site.