Summary

Bleeding disorders are conditions where the blood doesn't clot properly, leading to excessive or prolonged bleeding. Think of blood clotting as a complex cascade of proteins and cells working together to stop bleeding—when any part of this system is missing or not working properly, bleeding can be excessive or prolonged. Bleeding disorders can be inherited (hemophilia, von Willebrand disease) or acquired (liver disease, anticoagulants, DIC). The severity ranges from mild (only bleed with trauma or surgery) to severe (spontaneous bleeding, life-threatening hemorrhage). The key to management is recognizing the bleeding disorder (excessive bleeding, easy bruising, prolonged bleeding), identifying the cause (inherited vs acquired, which part of clotting system affected), assessing severity (how much bleeding, where), and providing appropriate treatment (replace missing factor, correct underlying cause, supportive care). Most bleeding disorders can be managed well with appropriate treatment, but severe bleeding can be life-threatening if not treated promptly.

Key Facts

• Definition: Conditions where blood doesn't clot properly
• Incidence: Varies by type (hemophilia rare, acquired common)
• Mortality: Low (<1%) unless severe bleeding
• Peak age: All ages (inherited = lifelong, acquired = any age)
• Critical feature: Excessive or prolonged bleeding
• Key investigation: Clinical assessment, coagulation tests (PT, APTT, platelets, specific factor tests)
• First-line treatment: Replace missing factor, correct underlying cause, supportive care

Clinical Pearls

"Think of it with excessive bleeding" — If a patient has excessive or prolonged bleeding (especially with minor trauma or surgery), think bleeding disorder. Don't miss this.

"PT vs APTT tells you where" — PT (prothrombin time) tests the extrinsic pathway. APTT (activated partial thromboplastin time) tests the intrinsic pathway. Which is abnormal tells you where the problem is.

"Inherited vs acquired matters" — Inherited disorders (hemophilia, von Willebrand) are lifelong. Acquired disorders (liver disease, anticoagulants) can be corrected by treating the cause.

"Severe bleeding is an emergency" — Severe bleeding, especially intracranial hemorrhage, is a medical emergency. Don't delay treatment.

Why This Matters Clinically

Bleeding disorders can cause life-threatening bleeding if not recognized and treated promptly. Early recognition (especially excessive bleeding), proper diagnosis (which part of clotting system affected), and appropriate treatment (replace factor, correct cause) are essential. This is a condition that haematologists and emergency clinicians manage, and prompt treatment can be life-saving.

Structured Approach: ABCDE

A - Airway

• Assessment: Usually patent (may be compromised if bleeding in airway)
• Action: Secure if compromised

B - Breathing

• Look: Usually normal (may have difficulty if significant blood loss)
• Listen: Usually normal
• Measure: SpO2 (usually normal)
• Action: Support if needed

C - Circulation

• Look: Signs of bleeding, shock
• Feel: Pulse (may be fast, weak), BP (may be low)
• Listen: Heart sounds (usually normal)
• Measure: BP (may be low), HR (may be fast)
• Action: Resuscitate if shock

D - Disability

• Assessment: Neurological status (may be altered if intracranial bleeding)
• Action: Assess if severe

E - Exposure

• Look: Full examination, look for bleeding sites
• Feel: Check for bleeding
• Action: Complete examination

Specific Examination Findings

Bleeding Assessment:

• Sites: Where is bleeding?
• Amount: How much bleeding?
• Type: Superficial (petechiae, mucosal) vs deep (joints, muscles)

Platelet vs Factor:

• Platelet problems: Petechiae, mucosal bleeding
• Factor problems: Deep bleeding, joint bleeding

Special Tests

Natural History (Without Treatment)

Untreated Bleeding Disorders:

• Severe bleeding: High risk
• Death: High risk if severe
• Poor outcomes: If not treated

Outcomes with Treatment

Factors Affecting Outcomes:

Good Prognosis:

• Early treatment: Better outcomes
• Mild disorder: Usually well managed
• Good compliance: Better outcomes
• Appropriate treatment: Better outcomes

Poor Prognosis:

• Delayed treatment: Higher risk of complications
• Severe disorder: More complications
• Poor compliance: Worse outcomes
• Inappropriate treatment: Worse outcomes

Prognostic Factors

Key Guidelines

1. WFH Guidelines (2020) — Guidelines for the management of hemophilia. World Federation of Hemophilia

Key Recommendations:

• Factor replacement
• Prevent bleeding
• Evidence Level: 1A

Landmark Trials

Multiple studies on factor replacement, outcomes.

Evidence Strength

What is a Bleeding Disorder?

A bleeding disorder is a condition where your blood doesn't clot properly, leading to excessive or prolonged bleeding. Think of blood clotting as a complex system working together to stop bleeding—when any part of this system is missing or not working properly, bleeding can be excessive or prolonged.

In simple terms: Your blood doesn't clot properly, so you bleed more than normal or for longer than normal. This can be mild (only with trauma) or severe (spontaneous bleeding), but with proper treatment, most people can manage it well.

Why does it matter?

Bleeding disorders can cause life-threatening bleeding if not recognized and treated promptly. Early recognition and appropriate treatment are essential. The good news? With proper treatment, most people can manage bleeding disorders well and live normal lives.

Think of it like this: It's like your blood's clotting system not working properly—it needs treatment to work right, but with the right treatment, most people do well.

How is it treated?

1. Diagnosis:

• Assessment: Your doctor will assess your bleeding and do blood tests
• Tests: You'll have tests to see which part of your clotting system isn't working
• Why: To see what's wrong and plan treatment

2. Treat Active Bleeding:

• If bleeding: Your doctor will control the bleeding and may give you the missing clotting factor or platelets
• Why: To stop the bleeding

3. Long-Term Treatment:

• If inherited (like hemophilia): You'll get the missing clotting factor when needed (for bleeding or to prevent bleeding)
• If acquired (caused by another condition): Your doctor will treat the underlying cause (like liver disease or stopping anticoagulants)
• Why: To prevent bleeding and manage the disorder

4. Prevent Bleeding:

• Avoid trauma: Be careful to avoid injuries
• Medications: Avoid medications that increase bleeding
• Why: To prevent bleeding episodes

The goal: Control bleeding when it happens, prevent bleeding, and help you live a normal life.

What to expect

Recovery:

• Active bleeding: Usually stops with treatment
• Long-term: Ongoing management (inherited) or treatment of cause (acquired)
• Quality of life: Usually good with appropriate treatment

After Treatment:

• Medicines: You may need to take medicines or get factor replacement when needed
• Monitoring: Regular monitoring of your clotting
• Lifestyle: Usually can live normally, but need to be careful to avoid injuries
• Follow-up: Regular follow-up to monitor your disorder

Recovery Time:

• Active bleeding: Usually stops quickly with treatment
• Long-term: Ongoing management

When to seek help

Call 999 (or your emergency number) immediately if:

• You're bleeding heavily
• You have a head injury and are bleeding
• You feel weak, dizzy, or faint from bleeding
• You have symptoms that concern you

See your doctor if:

• You have excessive or prolonged bleeding
• You bruise easily
• You have a known bleeding disorder and develop new symptoms
• You have concerns about bleeding

Remember: If you have excessive or prolonged bleeding, especially if you're bleeding heavily or have a head injury, call 999 immediately. Bleeding disorders are serious, but with proper treatment, most people can manage them well. Don't delay—if you're worried, seek help immediately.