Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure

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2017-09-01

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Abstract

Factor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors. CASE REPORT: A 17-year-old female with systemic lupus erythematosus and end-stage kidney disease secondary to Class IV lupus nephritis developed spontaneous subcutaneous and muscular hematomas and delayed major bleeding after invasive procedures. She had abnormal kaolin thromboelastography (kTEG; decreased maximal amplitude, representative of clot strength) initially attributed to thrombocytopenia and uremic platelet dysfunction, but her FXIII activity was undetectable, and a high-titer antibody against FXIII was identified. She had improvement in clinical bleeding and in kaolin thromboelastogram result and transient improvement in FXIII activity after each dose of plasma-derived FXIII concentrate (Corifact) or cryoprecipitate. Her inhibitor titers gradually improved with multiple immunosuppressive therapies and plasma exchange. While her FXIII activity level remained mildly decreased, she has not had additional significant bleeding. CONCLUSION: Treatment with either plasma-derived FXIII or cryoprecipitate is an effective treatment to normalize the kTEG variables and clinical bleeding diatheses associated with acquired FXIII inhibitors. Higher doses may be needed in patients with high-titer inhibitor.

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10.1111/trf.14185

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Rabik, Cara A, Meredith A Atkinson, Sangeeta Sule and John J Strouse (2017). Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure. Transfusion, 57(9). pp. 2159–2163. 10.1111/trf.14185 Retrieved from https://hdl.handle.net/10161/15607.

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Strouse

John J. Strouse

Associate Professor of Medicine

My research has focused on the epidemiology, risk factors, and prevention of the pulmonary and central nervous system complications of sickle cell disease and includes retrospective and prospective cohort studies and clinical trials.  I received my Ph.D. in clinical investigation from the Johns Hopkins Bloomberg School of Public Health for a series of studies to identify predictors of cognitive function in children with sickle cell disease.  This work has expanded to the evaluation of the interaction between environment and disease in both children and adults and the functional evaluation of adults with sickle cell disease.  My other research interests include the application of large clinical, research, and administrative databases to the study of rare hematological diseases and interventions to improve quality of and access to care for sickle cell disease. I serve on the American Society of Hematology Sickle Cell Taskforce and Sickle Cell Pain Guideline Panel and am co-chair of the American Society of Hematology Healthcare Professional Education and Training Work Group.

 


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