Successful Outcome of Severe Intra-cerebral Bleeding Associated with Acquired Factor V Inhibition: Utilization of Multiple Therapeutic Agents

Year 2018, Volume: 35 Issue: 1, 112 - 115, 01.01.2018

Panagiotis Andreadis Katerina Kafantari Aleka Agapidou Sofia Vakalopoulou Efthymia Vlachaki

Abstract

-Background: Acquired coagulation factor inhibitors
are antibodies that either inhibit activity or increase the
clearance of a clotting factor and lead to an increased
risk of bleeding. Most of the time, the disorder is
attributed to factor VIII inhibition (acquired haemophilia
A); however, other coagulation factors could also be
implicated.
Case Report: Herein, we report an interesting case
of a patient who underwent coronary artery bypass
grafting and received antibiotic treatment after surgery
with third generation cephalosporin. A month later,
he presented with extreme bleeding diathesis and
cerebral haemorrhage. Following a thorough clinical
and laboratory investigation, an acquired factor V
inhibitor was diagnosed. The patient received treatment
with corticosteroids, intravenous immunoglobulins,
anti-CD20 monoclonal antibodies (rituximab),
cyclophosphamide and recombinant factor VIIa. Finally,
despite the poor initial prognosis, the patient managed to
achieve a full recovery.
Conclusion: As there are no clear guidelines on acquired
coagulation inhibitor treatment, reports of such cases
could offer insight for future therapy choices. The case
was unique because the treatment regimen included a
combination

Keywords

Haemophilia, factor V, rituximab, inhibitor, cephalosporin

References

• 1. Boggio LN, Green D. Acquired hemophilia. Rev Clin Exp Hematol 2001;5:389-404.
• 2. W Collins P, Chalmers E, Hart D, Jennings I, Liesner R, Rangarajan S, et al. Diagnosis and management of acquired coagulation inhibitors: a guideline from UKHCDO. Br J Haematol 2013;162:758-73.
• 3. Huang JN, Koerper MA. Factor V deficiency: a concise review. Haemophilia 2008;14:1164-9.
• 4. Knöbl P, Lechner K. Acquired factor V inhibitors. Baillieres Clin Haematol 1998;11:305-18.
• 5. Kessler CM, Knöbl P. Acquired haemophilia: an overview for clinical practice. Eur J Haematol 2015;95(Suppl 81):36-44.
• 6. Franchini M, Lippi G. Acquired factor V inhibitors: a systematic review. J Thromb Thrombolysis 2011;31:449-57.
• 7. Ofosu FA, Crean S, Reynolds MW. A safety review of topical bovine thrombin-induced generation of antibodies to bovine proteins. Clin Ther 2009;31:679-97.
• 8. Wiwanitkit V. Spectrum of bleeding in acquired factor V inhibitor: a summary of 33 cases. Clin Appl Thromb Hemost 2006;12:485-8.
• 9. Motwani P, Howard L, Ali SS. Successful management of a possible antibiotic-related acquired factor V inhibitor: a case report and review of the literature. Acta Haematol 2013;129:182-4.
• 10. Hay CR, Brown S, Collins PW, Keeling DM, Liesner R. The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. Br J Haematol 2006;133:591-605.
• 11. Franchini M Castaman G, Coppola A Santoro C, Zanon E, Di Minno G, et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus 2015;13:498-513.