| Diagnosing AHA |
Awareness of the following features of AHA will aid recognition of the disease and facilitate referral of patients to specialist centers:
AHA is an acquired autoimmune disease that typically develops in middle age and beyond. Therefore, patients are typically older, with higher rates found in males.
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However, there is also a smaller peak in incidence among younger women (aged 20‐40 years) that is associated with pregnancy.
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Individuals who develop AHA have no previous or family history of bleeding
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; therefore, a lack of bleeding history does not rule out AHA. However, anticoagulant use is a frequent cause of bleeding in elderly individuals and should be ruled out. While joint bleeds are a hallmark of congenital hemophilia, they are rare in AHA.
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Therefore, an absence of joint bleeds does not rule out AHA. Instead, the bleeding pattern in AHA is characterized predominantly by subcutaneous bleeds, bleeding into other soft tissues or mucous membranes, and muscle hematomas.
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Bleeds can be severe (life, limb, or organ threatening) or mild enough that treatment is not needed.
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AHA is commonly associated with autoimmune disease, malignancy, infections, and drug‐related conditions.
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| Laboratory investigation |
If the effects of anticoagulant therapy can be ruled out as the cause of bleeding in a patient with unexpected hemorrhaging and no bleeding history, physicians should suspect AHA and investigate accordingly. An isolated, prolonged aPTT with a normal PT is usually the first indicator of AHA, even in patients without bleeding, and should always be investigated.
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Investigating an unusual aPTT should be prioritized along with (or even over) determining the site of bleeding.
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However, a prolonged aPTT may also be caused by coagulation factor deficiencies, von Willebrand disease, lupus anticoagulant, or therapy with anticoagulants
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; therefore, a differential diagnosis of AHA must be generated. In cases where there is a prolonged aPTT but it is difficult to generate a differential diagnosis, the prolonged aPTT results and details of bleeding should be referred to a hematology specialist.
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| Patient management: advice for nurses |
Examine the condition and integrity of the skin regularly and report any new appearances of bruising or skin breakdown to the hemophilia/hematology team immediately to aid monitoring of the bleeding episode and treatment decisions. Bleeds for which hemostatic treatment has been initiated should be frequently monitored to evaluate treatment efficacy. Patients with severe bruising have very fragile skin, and further subcutaneous bleeds can be induced by even routine manual handling and simple procedures. Modify patient interventions to ensure that physical care does not provoke or worsen bleeding: Where possible, avoid manual handling and use mechanical aids instead. Avoid non‐essential medical and nursing interventions that carry a bleeding risk. Avoid continuous electronic blood pressure monitoring, unless indicated by a clinically deteriorating condition. Use caution if venipuncture/cannulation is required; central venous access devices may be considered instead. Central venous catheter insertions must be performed with hemostatic coverage. Use caution when applying and removing adhesive dressings. Be aware that home visits and/or patient education may be required to help minimize injury risk in the patient's home. Be alert for signs of infection (which can be fatal in patients receiving immunosuppressive treatment to eradicate the anti‐FVIII inhibitor).
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