Monitoring

This section may serve as frameworks for how to monitor patients with AHA but need to be adapted based on clinical context and the availability of specialized laboratory analyses.

Acute Phase

Daily examination of the whole bodysuit is essential to identify new and follow ongoing bleeds and to evaluate the effectiveness of treatment.

  • Mark skin bleeds with a skin-safe marker.

  • Measure the extent of arm and/or leg involvement in extensive bleeding as there is a risk of compartment syndrome.

  • Central access e.g. central venous catheter or PICC line should be considered to facilitate a) blood sampling, b) intravenous treatment, and c) reduce the risk of skin bleeding caused by vein puncture.

  • If there is an obvious risk of infection (ongoing IST, elderly and/or fragile), consider administering antibiotic prophylaxis upon insertion.

Blood tests:

CRP, blood count, electrolytes, B-glucose are checked daily. APTT* and/or FVIII:C twice or more weekly. FVIII-inhibitors once weekly.

Fibrinogen and D-dimer; if concerns are raised for thrombotic microangiopathy caused by extensive treatment with activated factor concentrates with/without fibrinolysis inhibitors.

Vital signs:

Blood pressure, pulse, temperature daily. Weight two or more times per week.

Post acute phase (in hospital)

Bleeding is under control and IST has been initiated.

Blood test:

B-glucose, APTT*, FVIII:C and FVIII-inhibitors, CRP, blood count, and neutrophils** are checked once weekly. The frequency of testing depends on how the patient responds to prednisolone.

Vital signs:

Blood pressure, pulse, temperature daily. Weight two or more times per week.

Remission, complete treatment response to IST

Remission is achieved at FVIII:C > 0.50 IU/dL without measurable FVIII-inhibitors [1].

Blood test:

APTT* and/or FVIII:C, blood counts and neutrophils** should be checked monthly the first three months after the remission, then every third month. Monitoring ends one year after remission.

*APTT interferes with emicizumab. Emicizumab has a half-life of four weeks with expected detectable concentration long time after discontinuation.

**Patient on certain IST such as rituximab have a risk of neutropenia.