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Acquired haemophilia A is a haematologic emergency associated with a mortality rate of 9%-42%1,2,4

Early fatalities, within the first week, may result from gastrointestinal or lung bleeding, whereas later deaths may be caused by soft tissue bleeding such as intracranial or retroperitoneal bleeds2

European Registry Study5

  • 482 patients
  • 338 were treated
  • Median age 74 years
  • Mortality rate (based on number of patients treated): 19.5%

Survey6

  • 215 patients
  • Most patients >50 years
  • Mortality rate: 22.0%

UK Registry Study4,7

  • 172 patients
  • Median age 78 years
  • Mortality rate: 41.8% in 134 patients analysed for survival after starting immunosuppression treatment

Prompt diagnosis can reduce acquired haemophilia A mortality and morbidity

Effective treatments are available that can variously offer:8-10

  • High rates of haemostatic success
  • Low risk of thromboembolic events
  • Ability to objectively measure treatment response

Until the condition has been identified and treated, patients remain at risk of spontaneous, fatal bleeding, even if the initial presentation is mild4

If patients with undiagnosed acquired haemophilia A undergo surgery or invasive procedures, uncontrollable bleeding may result2

Lack of awareness can lead to diagnostic delays2

Patients with acquired haemophilia A can experience delays in referral and diagnosis ranging from days to months11,12

Data from the EACH2 registry showed that delayed time to treatment was associated with reduced responsiveness to treatment5

  • In 338 patients who received treatment for a bleeding episode, time to treatment varied significantly between those who responded to treatment and those who did not (median 1 vs 4 days; p=0.0155)5

Case study: acquired haemophilia A masked by anticoagulant treatment13


PATIENT CHARACTERISTICS

Age: 75 years

Sex: female

CLINICAL INVESTIGATIONS [normal range]

International normalised ratio: 2.49 [within therapeutic range]

Prothrombin time: 26.1 s [within therapeutic range]

aPTT: 73 s [23.0-32.0 s]

MEDICAL HISTORY [normal range]

Presentation: bruising and swelling on her chest, decreased mobility and progressing bilateral leg oedema despite no traumas; currently treating AF with anticoagulant

DEVELOPMENTS

Despite discontinuation of anticoagulant bleeding into the breast continued for several days and less than 25 mL of fluid could be aspirated from the haematoma, so a surgical decompression took place; 10 days post-surgery there was persistent bleeding and low hemoglobin necessitating transfusions

FURTHER CLINICAL INVESTIGATIONS

Factor VIII: 0.02 IU/mL (Day 10 after no improvement)

Factor VIII inhibitors: >200 BU (Day 10 after no improvement)

DIAGNOSIS

Acquired haemophilia A

The possibility of acquired haemophilia A in elderly patients on anticoagulant therapy should not be ignored

SUMMARY

Recognition saves lives

Acquired haemophilia A is a haematologic emergency4

Reduce mortality with a rapid response1-3

Lack of awareness can lead to diagnostic delays2