Rapid desensitization and subsequent immune tolerance induction in a patient with hypersensitivity and inhibitor to factor VIII

Development and desensitization therapy of high-response factor VIII inhibitors with severe allergic reaction in a moderate hemophilia A patient

“…Patients with hemophilia A are rarely to develop anti-FVIII allergic reaction coexisted with anti-FVIII inhibitors, which was reported to be induced either by FVIII itself or by other proteins in FVIII products only in a few case reports. 10–13 In this case, the patient was not allergic to rFVIII until inhibitor development. ITI therapy with pdFVIII lead to allergic reactions with elevated IgE levels and did not subside even after switching back to rFVIII suggesting that the allergic reaction could be caused by FVIII or also by other proteins like albumin or by both.…”

“…Desensitization protocol is the other inhibitor eradication strategy found effective in a few patients with allergic reactions. 10 In current case, patient achieved tolerance to FVIII through the desensitization protocol where it downregulates the expression of mast cells and basophils thereby inhibiting the release of inflammatory mediators (β-hexosaminidase, prostaglandins and leukotrienes), however the exact mechanism underlying this still remained elusive. 15 Further, the current report cannot be generalized as this was a single case report therefore the potential role of desensitization regimen in eliminating the inhibitor and allergic reaction to FVIII still need to be confirmed.…”