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Patient demographics

Incidence of acquired haemophilia A is approximately equal in men and women1-3

The condition usually affects older adults1,4

Median patient age is approximately 70 years4

Postpartum women may also be at increased risk3-5

Acquired haemophilia A may occur immediately after delivery, causing severe uterine or vaginal haemorrhage5

It may also develop up to 12 months after delivery, with extensive subcutaneous and mucosal bleeding as the most common symptoms5

In approximately 50% of cases in the EACH2 registry, acquired haemophilia A has no underlying cause3

The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients.

Five hundred and one (266 male, 235 female) patients from 117 centres and 13 European countries were included in the registry between 2003 and 2008.3

About half of acquired haemophilia A cases in the registry are idiopathic3

The other half of cases are associated with underlying conditions, such as autoimmune disease, pregnancy or malignancy3

Underlying disorders in the European Acquired Haemophilia (EACH2) registry3

Disorder Percentage of patients (N=501)
Idiopathic 51.9
Malignancy 11.8
Autoimmune disorders 13.4
Post partum 8.4
Infections 3.8
Drug induced 3.4
Dermatology 1.4
Other disorders 11.6

Case study: elderly patient6


PATIENT CHARACTERISTICS

Age: 90 years

Sex: female

CLINICAL INVESTIGATIONS [normal range]

PTT: 70.9 to >200 s [23.5-30.3 s]

Hemoglobin: 7.3 g/dL [12.0-16.0 g/dL]

Hematocrit: 21.9% [36.0-48.0%]

MRI: right gluteal and left thigh intramuscular haematomas

Factor VIII: <11% activity

Factor VIII inhibitors: 76 BU

MEDICAL HISTORY

Presentation: 2-week history of symptomatic anaemia and comprehensive purpuric lesions extending beyond the left arm and a haematoma on the left thigh

‘Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report’ by Rivera Cora NI, et al. This paper was published by BioMed Central under a Creative Commons Attribution (CC BY) license.

Recognising the clinical presentation of acquired haemophilia A “is paramount to prevent the occurrence of grave haemorrhagic episodes”

Case study: postpartum patient7


PATIENT CHARACTERISTICS

Age: 34 years

Sex: female

CLINICAL INVESTIGATIONS [normal range]

aPTT: 62 s [28-38 s]

Fibrinogen: 430 mg/dL [175-450 mg/dL]

Factor VIII: 3.5% activity [50-150%]

Factor VIII inhibitors: 10 BU

MEDICAL HISTORY

Presentation: 40 days following cesarean she developed progressively worsening spontaneous haematomas on both legs and intramuscular bleeding on the back, forearm, perimalleolar region and thighs

Cropped images from ‘Postpartum acquired hemophilia A: case report and literature review’ by Rodrigues DO, et al. This paper was published by Sci Forschen Inc. under a Creative Commons Attribution (CC BY) license.

Subcutaneous and intramuscular bleeding during the postpartum period could be signs of acquired haemophilia A

SUMMARY

Identifying those at risk

Elderly men and women1-4

Postpartum patients3-5

In a registry study, acquired haemophilia A was idiopathic in ~50% of cases3