Clotting Factor Deficiency (Hemophilia)Urgent

Known severe haemophilia A; painful swollen right knee after minor trauma (or groin pain with the hip held flexed, or a head knock); aPTT prolonged, PT and platelets normal

• Head injury, even minor, or new headache
• Neck swelling, stridor, dysphagia
• Groin pain with a flexed hip (iliopsoas)
• Tense forearm or calf with pain on passive stretch
• Haematemesis or melaena

• Von Willebrand disease
• Acquired haemophilia A (elderly, postpartum, autoimmune — prolonged aPTT that does not correct on mixing)
• Vitamin K deficiency / warfarin (PT prolonged)
• Heparin in the sample

• Admit: Head, neck, throat, iliopsoas, GI, forearm or calf bleeds, surgery, or bleeding not settling after the first dose
• ICU: Intracranial haemorrhage, airway-threatening bleed, haemorrhagic shock
• Transfuse: Red cells for symptomatic anaemia — factor is the haemostatic treatment

• Ask for the patient's haemophilia card first — type, severity, inhibitor status and usual dose.
• Severe = <1 IU/dL, moderate 1–5, mild 5–40; the aPTT can be normal in mild haemophilia.
• In India many states supply factor free through NHM-supported haemophilia treatment centres; emicizumab prophylaxis is expanding.
• An iliopsoas bleed mimics appendicitis or hip disease — groin pain with the hip held flexed, sometimes femoral nerve palsy.

• Delay factor for imaging in a head or neck bleed
• Give IM injections, NSAIDs or aspirin
• Aspirate a haemarthrosis routinely
• Give aPCC to a patient on emicizumab

• Waiting for CT before factor
• Wrong multiplier (0.5 for FVIII vs 1 for FIX)
• Missing an inhibitor when bleeding continues
• IM injections and NSAIDs

Ward or ICU by site; early physiotherapy after joint bleeds; haemophilia centre follow-up.