Time is Blood! Impact of Diagnostic Delays on Prognosis of Patients with Acquired Hemophilia A

When to be alert? Groups at increased risk

Acquired hemophilia A is a rare but serious acquired bleeding disorder characterized by the autoimmune formation of antibodies against endogenous coagulation factor VIII (FVIII). It is most commonly a disease of older people in connection with malignancy, infection, or another autoimmune condition, and in some cases, it is idiopathic. Another risk group for AHA includes pregnant women, as this disease can also occur during the postpartum period.

Clinical manifestations and diagnosis

Typical is the development of bleeding diathesis, often spontaneous. Bleeding is most often cutaneous, mucosal, but life-threatening internal bleeding can also occur. From the perspective of diagnosis, time is a crucial factor – doctors must consider this disease as part of the differential diagnosis. Laboratory proof is then relatively simple – it’s characterized by an isolated prolongation of activated partial thromboplastin time (aPTT), and a specialized laboratory will subsequently confirm a decrease in FVIII levels and the presence of its inhibitor.

Findings from a retrospective study

The retrospective study quoted below by a team of American experts evaluated data within one hospital system from March 2010 to January 2017. Six patients meeting the criteria for AHA were identified. The median age was 79.5 years, and the median time to diagnosis from the first symptoms was 14 days. Three of these patients had cancer, and for the other three, no triggering cause was identified.

One patient died before starting bypass treatment, while the remaining 5 were initially treated with recombinant activated FVII, and 2 of them received recombinant porcine FVIII in the second line due to refractory bleeding. Effective hemostasis was achieved in all patients; however, all 5 died in the following 5 years – 4 due to bleeding complications.

Conclusion

The authors conclude that the key to treatment and prognosis lies in the earliest possible correct diagnosis. AHA is generally associated with high mortality. In the diagnostic algorithm of unexplained bleeding and isolated prolonged aPTT, a quick mixing test can be performed, where no correction of prolongation occurs after adding normal plasma. This is a crucial step towards suspecting this disease and performing all necessary tests, either on-site or at a specialized hematology center. Prompt diagnosis increases the chance of managing bleeding symptoms and can save lives.

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Source: Gragner M., Imbo B., Hobson J. et al. Time is blood: the impact of diagnostic delays on acquired hemophilia A. Cureus 2022 Feb 9; 14 (2): e22048, doi: 10.7759/cureus.22048.