SESSION TITLE: Miscellaneous Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: Among the autoimmune hemorrhaphilias, acquired Factor XIII (FXIII) deficiency is one of the rarest. The majority of patients are elderly, with a median age between 60-70 years old. We present a case of a young woman with a severe bleeding diathesis due to an inhibitor to FXIII from systemic lupus erythematosus (SLE).
CASE PRESENTATION: A 23-year-old Taiwanese female with a history of diffuse large B-cell lymphoma presented to the hospital with spontaneous bilateral hip and thigh hematomas, menorrhagia, and epistaxis. Platelets and routine coagulation studies were all normal, so FXIII levels were measured and discovered to be absent. A 50:50 mixing study confirmed the diagnosis of an inhibitor rather than a deficiency. Initially, her autoimmune hemorrhaphilia was presumed to be secondary to her previous lymphoma treatment. However, the patient failed to improve despite aggressive treatment with CyBorD, IVIG, rituxumab, cryoprecipitate, and steroids. She was re-hospitalized due to further bleeding complications, including partial blindness from hemorrhagic posterior vitreous detachment, and transferred to the MICU for presumed TRALI requiring intubation. In total, she consumed 340 units of cryoprecipitate, 17 units of Factor VII, 12 units of packed red cells, and 12 units of platelets over the course of 29 days. During this time, the patient was also diagnosed with SLE and started on high-dose Cytoxan, Solumedrol, and Plaquenil. After this intervention, her functional Factor XIII assay improved dramatically from 9% to 70% and she was eventually discharged home.
DISCUSSION: As of 2011, only 37 cases of acquired FXIII deficiency have been reported in the literature, and only 2 of them from SLE. Most cases were related to lymphoproliferative disorders, chronic renal or liver failure, and drugs such as isoniazid, penicillin, phenytoin, or practolol. A deficiency of functional FXIII (either congenital or accquired) classically presents as spontaneous severe intramuscular or soft tissue bleeding, retroperitoneal bleeding, or intracranial hemorrhage, which is the most common cause of death. Treatment is aimed on three fronts: increasing FXIII activity, inhibitor elimination, and removal of the offending cause. Due to the presence of an inhibitor, replacement therapy with FFP and cryoprecipitate is often unsuccessful. Administration of FXIII concentrate is more effective, but prohibitively expensive for most institutions. Inhibitor levels can be reduced via IVIG or immunosuppressive agents.
CONCLUSIONS: In our patient, significant progress in raising her Factor XIII activity levels was only achieved after discovering and aggressively treating the underlying cause, which, in retrospect, was most likely her SLE rather than her history of lymphoma.
Reference #1: Luo et al. Haemophilia 2011;17(3):393-398
DISCLOSURE: The following authors have nothing to disclose: Ian Lee, Jo Persoon-Gundy, Ching-Fei Chang
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