The adequate performance and correct interpretation of assays for coagulation factor inhibitors play a critical role for the hemostasis laboratory. Both, false positive and false negative inhibitor assays may be reported, leading to erroneous patient’s management. Therefore, we decided to present a case with a spurious image of an exceptionally rare acquired combined haemophilia A, B and C, with severe factor ( F) VIII, IX and XI deficiency, associated with high titre anti - F VIII, IX and XI inhibitors in a 4 years old girl with Henoch-Schönlein type vasculitis. Finally, performing, beside coagulometric methods also antigenic ELISA assays, we had to invalidate the diagnosis. The performance of antiphospholipd antibodies clarified the diagnosis , finally concluding as definite diagnosis Transient Lupus Anticoagulant Syndrome, with decisive impact on therapy and follow-up.
8. Singh AK, Rao KP, Kizer J, Lazarchick J.Lupus anticoagulants in children. Ann Clin Lab Sci. 1988 Sep- Oct;18(5):384-7
9. Sreedharanunni S, Ahluwalia J, Kumar N, Bose SK, Dhawan R, Malhotra P. Lupus anticoagulant-hypoprothrombinemia syndrome: a rare cause of intracranial bleeding. Blood Coagul Fibrinolysis. 2016 Dec;10-1097.
10. Sarker T, Roy S, Hollon W, Rajpurkar M. Lupus anticoagulant acquired hypoprothrombinemia syndrome in childhood: two distinct patterns and review of the literature.Haemophilia. 2015 Nov;21(6):754-60.
11. Knobe K, Tedgård U, Ek T, Sandström PE, Hillarp A. Lupus anticoagulants in two children-bleeding due to nonphospholipid-dependent antiprothrombin antibodies. Eur J Pediatr. 2012 Sep;171(9):1383-7.
12. Carvalho C, Viveiro C, Maia P, Rezende T. Acquired antiprothrombin antibodies: an unusual cause of bleeding. BMJ Case Rep. Published online 2013 Jan; doi:
13. A.Taher, R.Abiad, I.Uthman. Coexistence of lupus anticoagulant and acquired haemophilia in a patient with monoclonal gammopathy of unknown significance. Lupus 2003; 12(11):854-6.
14. J.D.Dreisbach, L.P.Dreisbach, D.E.Young, P.B.Dreisbach. Acquired factor VIII inhibitor and lupus anticoagulant presenting with prolonged aPTT: a case report. Grand Rounds 2010,10, 19-24.
15. G.W.Moore. Current Controversies in Lupus Anticoagulant Detection.Antibodies 2016, 5(4), 22.
16. Jin J, Zehnder JL. Prozone effect in the diagnosis of Lupus Anticoagulant for the Lupus Anticoagulant-Hy poprothrombinemia Syndrome.Am J Clin Pathol. 2016 Aug;146(2):262-7.
17. Adcock DM, Favaloro EJ. Pearls and pitfalls in factor inhibitor assays.Int J Lab Hematol. 2015 May; 37Suppl 1:52-60.
18. Doma SA, Hillarp A, Pajič T, Andoljšek D, Černelč P, PreldžnikZupan I. Concurrent acquired inhibitors to factor VIII and IX, a laboratory artifact: a case report. Biochem Med (Zagreb). 2016 Jun; 26(2): 279-284.
19. Fletcher M, Crombet O, Morales-Arias.J. Successful treatment of acquired hemophilia A with rituximab and steroids in a 5-year-old girl.J PediatrHematol Oncol. 2014 Mar;36(2):103-4.
20. Yang C, Yu Z, Zhang W, Cao L, Su J, Sha P, Bai X, Wang Z, Ruan C. A single-center clinical study of 22 patients with acquired hemophilia. ZhonghuaXue Ye XueZaZhi. 2015 Feb;36(2):107-11.
21. Wermes C, Niekrens C, Sykora KW. Successful longtime treatment with mycophenolate-mofetil in a child with acquired factor VIII inhibitor. Hamostaseologie. 2012 Jul; 32 Suppl 1:75-8.
22. Joly B, d’Oiron R, Desconclois C, Bendelac L, Rafowicz A, Meyzer C, Labrune P, Veyradier A.Mild hemophilia A fortuitously discovered during Henoch-Schonleinpurpura. Arch Pediatr. 2015 Nov;22(11):1167-70.
23. Gration B, Osakwe E.Self-limiting recurrent bullous Henoch- Schonleinpurpura with lupus anticoagulant.BMJ Case Rep. Published online 2015 Jan. doi: