Careful Consideration Needed for Management of Acquired (Autoimmune) Hemophilia

Acquired (autoimmune) hemophilia (AHA) is a rare and serious bleeding disorder caused by autoantibodies that target coagulation factor VIII. This condition can be life-threatening due to spontaneous bleeding and is often diagnosed in adults, particularly in older people. Understanding its demographics, outcomes, and readmission rates is crucial for improving patient care and management, according to research published this month in Blood Vessels, Thrombosis & Hemostasis.

Despite significant advancements in therapy elements, there is limited evidence regarding the epidemiology and outcomes of these AHA treatments in a broader population. Aditi Sharma, from the Karmanos Cancer Institute at Wayne State University School of Medicine, and colleagues aimed to shed light on the epidemiology of AHA, including readmission rates and causes of readmission, through a nationwide population-based retrospective analysis.

Readmission rates for patients with AHA are affected by the recurring nature of bleeding episodes and the necessity for ongoing treatment and monitoring. Patients frequently require multiple hospitalizations to manage acute bleeds and complications associated with immunosuppressive therapy.

Strategies to lower readmission rates include careful monitoring, patient education, and coordinated care between hematologists and primary care providers.

The authors pointed out that in elderly populations with multiple additional medical comorbidities, it is crucial to be aware of the treatment-related consequences, such as infections, thrombosis, and recurrent bleeding diathesis. Mortality rates can be high due to severe bleeding complications, especially among the elderly. However, with appropriate and timely treatment, many patients can achieve remission and control of bleeding episodes.

AHA occurs at a low rate of 1.5 cases per million people annually and mainly impacts older adults, with an average age of diagnosis around 70 years. Both men and women can be affected, though it is slightly more common in men. The condition may be linked to several underlying factors, such as autoimmune disorders, cancers, and pregnancy in younger women.

Treatment generally involves two primary approaches: controlling bleeding and eliminating autoantibodies. Acute bleeding is managed with hemostatic agents such as recombinant factor VIIa, activated prothrombin complex concentrate, or Hemlibra (emicizumab). To eradicate autoantibodies, immunosuppressive therapies, including corticosteroids, cyclophosphamide, and rituximab, are used.

The study analyzed 1,450 admissions for AHA from 2016 to 2019. Common comorbidities on admissions included autoimmune disease (13.5%), solid malignancy (20.9%), and hematologic malignancy (3.9%). The researchers claimed that the higher rate of malignancies was due to their inability to distinguish active malignancies from past malignancies.

Complications observed on initial admission to the hospital included bleeding (30.2%), thrombotic events (17.2%), acute myocardial infarction (9.5%), and venous thromboembolism (4.4%). The 30-day readmission rate of 27% was attributed mainly to infections (30.8%) and bleeding (28.2%), with a 10.8% mortality rate during readmissions compared with a 7% inpatient mortality rate upon the initial visit. Higher readmission rates were also observed in patients with autoimmune diseases (35.5% vs. 23.3% without autoimmune disease). ​

Since more than a quarter of hospital discharges were readmitted, Sharma and team noted that this result further demonstrated the complexity of challenges in managing AHA. Additionally, the high infection rates are most likely due to continued immunosuppressive therapy, and the high bleeding rates suggest inadequate autoantibody clearance.

The study emphasizes the considerable impact of AHA on the U.S. healthcare system, examining demographics, complications during hospitalization, readmission rates, and mortality. The elevated 30-day readmission rates were primarily attributed to infections and bleeding, with significant inpatient and readmission mortality, along with the presence of comorbidities and complications. A more cautious strategy for factor replacement to reduce thrombotic complications in AHA is necessary.