Activity Thresholds: WFH definitions based on baseline factor (VIII/IX) activity. While lab results define the class, the clinical phenotype determines treatment strategy.
Factor Activity (%)
Guidelines & Evidence
Clinical Details
Section 1
When to Use
When to Use
Classifying the severity of newly diagnosed Haemophilia A (Factor VIII deficiency) or Haemophilia B (Factor IX deficiency).
Correlating laboratory factor activity with clinical bleeding phenotype.
Guiding the necessity for primary prophylaxis vs. episodic (on-demand) treatment.
Patient Population
Adults and children with a baseline deficiency of factor VIII or IX.
Section 2
Formula & Logic
Activity Thresholds
Severity
Factor Level (% or IU/mL)
Clinical Phenotype
Severe
< 1% (< 0.01)
Frequent spontaneous bleeds into joints or muscles; often diagnosed in infancy.
Moderate
1 - 5% (0.01 - 0.05)
Few spontaneous bleeds; prolonged bleeding with minor trauma or surgery.
Mild
6 - 40% (0.06 - 0.40)
No spontaneous bleeding; hemorrhage only with significant trauma or major surgery.
Section 3
Pearls/Pitfalls
The 'Invisible' Severe
While laboratory thresholds are standard, some patients with < 1% activity have a "moderate" clinical phenotype, while others with 2-3% activity bleed spontaneously like "severe" patients. Clinical phenotype should ultimately guide prophylaxis decisions.
Emicizumab Impact
In patients with severe Haemophilia A, Emicizumab prophylaxis can transition the bleeding clinical phenotype from "Severe" to effectively "Mild," allowing for much greater physical activity.
Section 4
Evidence Appraisal
Primary Strategy
WFH Guidelines for the Management of Hemophilia, 3rd edition.
Srivastava A et al. • Haemophilia. 2020;Global consensus on severity thresholds.
These thresholds have been the foundation of haemophilia care since the 1950s, refined by the ISTH in 2001 to ensure uniform language in global clinical trials.
Last Comprehensive Review: 2026
Guidelines & Evidence
Clinical Details
Section 1
When to Use
When to Use
Classifying the severity of newly diagnosed Haemophilia A (Factor VIII deficiency) or Haemophilia B (Factor IX deficiency).
Correlating laboratory factor activity with clinical bleeding phenotype.
Guiding the necessity for primary prophylaxis vs. episodic (on-demand) treatment.
Patient Population
Adults and children with a baseline deficiency of factor VIII or IX.
Section 2
Formula & Logic
Activity Thresholds
Severity
Factor Level (% or IU/mL)
Clinical Phenotype
Severe
< 1% (< 0.01)
Frequent spontaneous bleeds into joints or muscles; often diagnosed in infancy.
Moderate
1 - 5% (0.01 - 0.05)
Few spontaneous bleeds; prolonged bleeding with minor trauma or surgery.
Mild
6 - 40% (0.06 - 0.40)
No spontaneous bleeding; hemorrhage only with significant trauma or major surgery.
Section 3
Pearls/Pitfalls
The 'Invisible' Severe
While laboratory thresholds are standard, some patients with < 1% activity have a "moderate" clinical phenotype, while others with 2-3% activity bleed spontaneously like "severe" patients. Clinical phenotype should ultimately guide prophylaxis decisions.
Emicizumab Impact
In patients with severe Haemophilia A, Emicizumab prophylaxis can transition the bleeding clinical phenotype from "Severe" to effectively "Mild," allowing for much greater physical activity.
Section 4
Evidence Appraisal
Primary Strategy
WFH Guidelines for the Management of Hemophilia, 3rd edition.
Srivastava A et al. • Haemophilia. 2020;Global consensus on severity thresholds.
These thresholds have been the foundation of haemophilia care since the 1950s, refined by the ISTH in 2001 to ensure uniform language in global clinical trials.