Acquired factor VIIIC deficiency due to circulating factor VIIIC inhibitors

Area of Science:

• Hematology
• Immunology

Background:

• Acquired inhibitors to coagulation Factor VIII (Factor VIIIC) present a significant management challenge in non-hemophilic individuals.
• These inhibitors cause bleeding diatheses, necessitating individualized treatment strategies.

Observation:

• Three adult patients without hemophilia were identified with acquired Factor VIIIC inhibitors.
• One patient had a history of rheumatoid arthritis; another had received phenylbutazone for arthralgias.
• The third patient had no apparent underlying condition preceding inhibitor development.

Findings:

• Two patients received Prednisolone treatment.
• This immunosuppressive therapy resulted in rapid clinical improvement.
• Factor VIIIC activity normalized in both patients treated with Prednisolone.

Implications:

• Immunosuppressive therapy, particularly with Prednisolone, appears effective in managing acquired Factor VIIIC inhibitors in non-hemophilic adults.
• This approach offers a potential therapeutic strategy for this rare but serious bleeding disorder.
• Further research into the role and rationale of immunosuppression is warranted.