Acquired FXIII Deficiency in a Patient with Systemic Lupus Erythematodes: Clinical Presentation and Management

Abstract number: P1311

Schlammadinger¹ Á, AjznerMéhes²¹ ÉL, Papp¹ M, Muszbek² L, Udvardy¹ M, Boda¹ Z

¹¹2^nd Department of Medicine, University of Debrecen, Debrecen, Hungary ¹¹2^nd Department of Medicine, University of Debrecen, Debrecen, Hungary ²¹¹2^nd Department of Medicine, University of Debrecen, Debrecen, Hungary²Clinical Research Center, University of Debrecen, Debrecen, Hungary ²²Clinical Research Center, University of Debrecen, Debrecen, Hungary

Acquired FXIII deficiency is a rare, but potentially life threatening bleeding disorder. No accepted therapeutic recommendations are available. A 28 year old female patient with a history of systemic lupus erythematodes and lupus nephritis was referred to our department because of large spontaneous subcutaneous haematomas. The clinical presentation and the underlying autoimmun disease suggested the presence of an acquired coagulation factor inhibitor. However, all coagulation times were normal as was the platelet count and were the platelet function tests. On the other hand, the clot dissolved in 5 M urea within 2 hours. FXIII activity was below 1%. As transfusional therapy was limited because of IgA deficiency, activated FVII (FVIIa) was used to control the bleeding. FVIIa doubled the time needed for the dissolution of the clot in the clot solubility test and in thromboelastography. Intensive immunosuppressive therapy with steroid, cyclophosphamide and cyclosporine was ineffective. One round of plasmapheresis with albumin supplementation and cautious immunoglobulin therapy (3 × 0.4 mg/kg) were also without effect. Immunosuppression was completed with an anti-CD20 monoclonal antibody, rituximab. On the twelfth day 3750 U (62.5 U/bwkg) Fibrogammin was used to control the growth of a haematoma. Although no further transfusions were needed, FXIII levels remained below 1% and no improvement in the patient's condition was seen. Two other Fibrogammin injections (4000 U and 1000 U) were administered to achieve immunotolerance. As no sustained rise in FXIII level was observed, plasmapheresis with fresh frozen plasma was carried out twice. A rapid increase in FXIII level (to 20–25%) followed, the patient's disabling pain subsided, the size of the haematomas substantially reduced. In the past 5 months she had no relapses. The combined administration of plasmapheresis and immunosuppression together with FXIII supplementation seems to be an effective treatment for acquired FXIII deficiency.