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Haemophilia

Also known as: haemophilia A · haemophilia B · factor VIII deficiency · factor IX deficiency

An X-linked inherited deficiency of clotting factor VIII (haemophilia A) or IX (haemophilia B) causing a bleeding tendency — spontaneous or trauma-related bleeds into joints (haemarthrosis) and muscles, and prolonged bleeding after injury or surgery.

Source · Reviewed 2026-08-15 · v1.0— AI-assisted draft — verify against the cited guideline before clinical use.

⚕️ Education only — not medical advice. Verify against the latest official guideline and your local protocol; confirm every drug dose against the cited source before clinical use.

Who gets it

Males (X-linked), with female carriers; a family history is common but about a third are new mutations; severity depends on the factor level.

Red flags — escalate / refer

Intracranial, throat/neck or gastrointestinal bleeding, or a large muscle/compartment bleed → emergency factor replacement FIRST (don't delay for imaging).

Work-up / diagnosis

1A prolonged APTT with a normal PT and platelets; confirm with specific factor assays (VIII/IX) and genetic testing.
2Assess severity and screen for inhibitors in treated patients.

Management — overview

Specialist haemophilia-centre care — replace the missing factor (factor concentrate) promptly for bleeds and to cover surgery.
Adjuncts — desmopressin for mild haemophilia A, tranexamic acid for mucosal bleeds (avoid in haematuria); prophylactic factor to prevent joint damage in severe disease.
AVOID intramuscular injections and aspirin/NSAIDs; treat early ('treat first, image later'); manage inhibitors; genetic counselling.

In-depthFull dosing & advanced protocol

Drug dosing

Clotting-factor concentrate (VIII or IX)

Bleeds and surgical cover

Adult dose: Per protocol and severity; prophylaxis in severe disease
Route: Intravenous

Caution: Avoid IM injections and NSAIDs/aspirin; watch for factor inhibitors.

ICMRStandard Treatment Workflows (STWs), India — Dept. of Health Research & ICMR (2022+) ↗

Desmopressin (mild A) / tranexamic acid

Mild haemophilia A; mucosal bleeds

Adult dose: Per protocol
Route: IV / oral

WHOWHO Model List of Essential Medicines (electronic EML) (2025 (24th)) ↗

When in doubt, treat first

Give factor before imaging for a serious bleed — don't wait.
Avoid IM injections, aspirin and NSAIDs.
Prophylaxis prevents arthropathy in severe disease; watch for factor inhibitors.

ICMRStandard Treatment Workflows (STWs), India — Dept. of Health Research & ICMR (2022+) ↗

References

Guidelines change. If something here is out of date, tell us — we keep these current.

🚩 Report outdated / suggest update

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👤 Who gets it

🚩 Red flags — escalate / refer

🔎 Work-up / diagnosis

🧭 Management — overview

💊 Drug dosing

Clotting-factor concentrate (VIII or IX)

Desmopressin (mild A) / tranexamic acid

📈 When in doubt, treat first

📚 References

More protocols

👤 Who gets it

🚩 Red flags — escalate / refer

🔎 Work-up / diagnosis

🧭 Management — overview

💊 Drug dosing

Clotting-factor concentrate (VIII or IX)

Desmopressin (mild A) / tranexamic acid

📈 When in doubt, treat first

📚 References

More protocols

Who gets it

Red flags — escalate / refer

Work-up / diagnosis

Management — overview

Drug dosing

When in doubt, treat first

References

Who gets it

Red flags — escalate / refer

Work-up / diagnosis

Management — overview

Drug dosing

When in doubt, treat first

References