Summary

A haemolytic crisis is rapid destruction of red blood cells (hemolysis), leading to anemia, jaundice (yellowing from bilirubin), and potentially serious complications. Think of red blood cells as having a normal lifespan of about 120 days—in hemolysis, red cells are destroyed prematurely, faster than the bone marrow can replace them, leading to anemia. Hemolysis can be caused by many things: autoimmune (immune system attacks own red cells), mechanical (artificial heart valves, microangiopathy), infections (malaria, certain bacteria), drugs, or inherited conditions (sickle cell disease, G6PD deficiency). The severity ranges from mild (minimal symptoms) to severe (rapid anemia, shock, renal failure). The key to management is recognizing hemolysis (anemia, jaundice, dark urine, sometimes fever), confirming the diagnosis (blood tests showing hemolysis—low hemoglobin, high bilirubin, high LDH, low haptoglobin, reticulocytosis), identifying the cause (autoimmune, mechanical, infection, drug, inherited), and providing appropriate treatment (treat underlying cause, blood transfusion if severe anemia, supportive care). Early recognition and prompt treatment are essential—severe hemolysis can cause rapid anemia, shock, and renal failure.

Key Facts

• Definition: Rapid destruction of red blood cells
• Incidence: Rare to uncommon (varies by cause)
• Mortality: Low (<1%) unless severe and untreated
• Peak age: All ages (varies by cause)
• Critical feature: Anemia, jaundice, signs of hemolysis
• Key investigation: Blood tests (FBC, bilirubin, LDH, haptoglobin, reticulocytes, Coombs test)
• First-line treatment: Treat underlying cause, blood transfusion if severe, supportive care

Clinical Pearls

"Anemia + jaundice = hemolysis until proven otherwise" — If a patient has anemia and jaundice (yellowing), think hemolysis. This is a classic combination.

"LDH up, haptoglobin down = hemolysis" — LDH (lactate dehydrogenase) is elevated in hemolysis. Haptoglobin is low (consumed binding free hemoglobin). This pattern is diagnostic.

"Coombs test tells you if autoimmune" — The Coombs test (direct antiglobulin test) tells you if hemolysis is autoimmune (positive) or not (negative). This guides treatment.

"Treat the cause" — Hemolysis is a symptom, not a disease. Always look for and treat the underlying cause (autoimmune, infection, drug, etc.).

Why This Matters Clinically

Hemolytic crisis can cause rapid anemia, shock, and renal failure if not recognized and treated promptly. Early recognition (especially anemia + jaundice), prompt diagnosis (hemolysis tests), identification of cause, and appropriate treatment are essential. This is a condition that haematologists and emergency clinicians manage, and prompt treatment prevents complications.

Structured Approach: ABCDE

A - Airway

• Assessment: Usually patent
• Action: Secure if compromised

B - Breathing

• Look: May have respiratory distress (anemia)
• Listen: Usually normal
• Measure: SpO2 (may be low if severe anemia)
• Action: Support if needed

C - Circulation

• Look: Signs of anemia, shock
• Feel: Pulse (may be fast, weak), BP (may be low)
• Listen: Heart sounds (may have flow murmur if anemia)
• Measure: BP (may be low), HR (may be fast)
• Action: Resuscitate if shock

D - Disability

• Assessment: Usually normal (may be altered if severe)
• Action: Assess if severe

E - Exposure

• Look: Full examination, look for jaundice, splenomegaly
• Feel: Spleen, liver
• Action: Complete examination

Specific Examination Findings

Hematological Examination:

• Pallor: Anemia
• Jaundice: Yellow skin/eyes
• Splenomegaly: Extravascular hemolysis

Organ Examination:

• Spleen: May be enlarged
• Liver: Usually normal (may be enlarged if cause)

Special Tests

Natural History (Without Treatment)

Untreated Hemolytic Crisis:

• Severe anemia: Almost certain if severe
• Shock: High risk if severe
• Poor outcomes: If not treated

Outcomes with Treatment

Factors Affecting Outcomes:

Good Prognosis:

• Early treatment: Better outcomes
• Mild-moderate: Usually recovers quickly
• Treatable cause: Better outcomes
• No complications: Better outcomes

Poor Prognosis:

• Delayed treatment: Higher risk of complications
• Severe hemolysis: Longer recovery
• Untreatable cause: Worse outcomes
• Complications: Worse outcomes

Prognostic Factors

Key Guidelines

1. BSH Guidelines (2017) — Guidelines for the diagnosis and management of adult autoimmune haemolytic anaemia. British Society for Haematology

Key Recommendations:

• Identify cause
• Treat underlying cause
• Evidence Level: 1A

Landmark Trials

Multiple studies on treatment, outcomes.

Evidence Strength

What is a Hemolytic Crisis?

A hemolytic crisis is when your red blood cells are being destroyed too quickly, faster than your body can replace them. Think of red blood cells as having a normal lifespan—in hemolysis, they're destroyed prematurely, leading to anemia (not enough red cells) and jaundice (yellowing from the breakdown products).

In simple terms: Your red blood cells are being destroyed too quickly. This causes anemia (not enough red cells) and jaundice (yellowing), but with proper treatment, most people recover well.

Why does it matter?

Hemolytic crisis can cause rapid anemia, shock, and renal failure if not recognized and treated promptly. Early recognition and appropriate treatment are essential. The good news? With proper treatment, most people recover well.

Think of it like this: It's like your red blood cells being destroyed too quickly—it needs treatment to stop this and help your body recover, but once treated, most people recover well.

How is it treated?

1. Diagnosis:

• Assessment: Your doctor will assess you and do blood tests
• Tests: You'll have tests to confirm that your red cells are being destroyed and to find the cause
• Why: To confirm the diagnosis and see what's causing it

2. Treat the Cause:

• If autoimmune: You'll get medicines to suppress your immune system (usually steroids)
• If infection: You'll get treatment for the infection
• If drug: You'll stop the drug
• If mechanical: Your doctor will treat the underlying cause (like a heart valve problem)
• If inherited: You'll get supportive care
• Why: To stop the red cell destruction

3. Support Your Body:

• Blood transfusion: If you're very anemic, you'll get a blood transfusion
• Why: To support your body while the cause is treated
• Other support: You may get other supportive care as needed

The goal: Stop the red cell destruction (treat the cause), support your body (blood transfusion if needed), and help you recover.

What to expect

Recovery:

• Treatment: Usually starts immediately
• Recovery: Usually starts improving within days to weeks
• Full recovery: Most people recover well with treatment

After Treatment:

• Medicines: You may need to take medicines (if autoimmune, etc.)
• Monitoring: Your doctor will monitor to make sure you're recovering
• Follow-up: Regular follow-up to monitor your recovery

Recovery Time:

• Acute phase: Usually days to weeks
• Long-term: Depends on cause (may need ongoing treatment if chronic)

When to seek help

Call 999 (or your emergency number) immediately if:

• You feel very weak, dizzy, or faint
• You have severe breathlessness
• You feel very unwell
• You have symptoms that concern you

See your doctor if:

• You have unexplained fatigue or weakness
• You notice your skin or eyes are yellow (jaundice)
• You have dark urine
• You have symptoms that concern you

Remember: If you have unexplained fatigue or weakness, especially if you also have yellowing of your skin or eyes, see your doctor. Hemolytic crisis is serious, but with proper treatment, most people recover well. Don't delay—if you're worried, seek help immediately.