Measurement of factor XIII levels, followed by replacing deficiencies if found, may help to achieve sustained hemostasis in the management of bleeding in patients with acquired hemophilia A.
Jameel Abdulrehman, MD, of the University of Toronto in Ontario and his colleagues retrospectively reviewed cases of acquired hemophilia A with bleeding at a large hospital from 2015-2016. The findings were published in a letter to the editor in Haemophilia.
The researchers reported that depletion of Factor XIII (FXIII) in the setting of acquired hemophilia A has only been reported in a single case. In the present analysis, the researchers identified a total of seven patients with acquired hemophilia A from 2015-2016. FXIII antigen levels were measured in five cases and were found to be low in four cases, and three patients required FXIII replacement to secure hemostasis.
The relationship between acquired hemophilia A and FXIII deficiency was evaluated using descriptive case analysis.
After analysis, the team reported that measuring FXIII levels may be useful in refractory patients, in whom bleeding remains uncontrolled despite the use of standard therapy. Additionally, the results supported FXIII supplementation if a deficiency is detected.
“FXIII replacement was not complicated by any infusion reactions or thromboembolic events,” they wrote.
The replaced FXIII levels dropped more rapidly than expected, the researchers noted. The established half-life of pd-XIII is 6.6 days plus or minus 2.29 days; however, in the most severe of the cases reported, it was depleted “within a few days,” they wrote. “This is compatible with either consumption of FXIII or inhibition/clearance by an autoantibody.”
Dr. Abdulrehman and his colleagues acknowledged that FXIII deficiency can be challenging to identify because the majority of coagulation assays do not measure FXIII levels.
“As we gain a greater appreciation for the role of FXIII in various pathological states, access to accurate FXIII testing will become increasingly important,” they added.
No funding sources were reported. The authors reported having no conflicts of interest.
SOURCE: Abdulrehman J et al. Haemophilia. 2019 Mar 7. doi: 10.1111/hae.13690.