https://doi.org/10.4081/btvb.2023.53

A case of acquired factor XIII deficiency secondary to plasmablastic lymphoma

Vol. 2 No. 1 (2023)
Submitted: 25 October 2022
Accepted: 20 February 2023
Published: 9 March 2023
Factor XIII, Bleeding, Myeloma
Abstract Views: 417
PDF: 272
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Authors

  • Vincenzo Sammartano Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Adele Santoni Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Elisabetta Zappone https://orcid.org/0000-0002-4276-0214 Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Paola Calzoni Coagulative Disorders Laboratory Unit, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Daniela Fineschi Coagulative Disorders Laboratory Unit, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Eleonora Franceschini Coagulative Disorders Laboratory Unit, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Federico Caroni Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Anna Sicuranza Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Monica Bocchia Hematology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, “Santa Maria alle Scotte” University Teaching Hospital of Siena, Italy.
  • Luca Puccetti • luca.puccetti@unisi.it Hemostasis and Thrombosis Unit, “Santa Maria alle Scotte” University Teaching Hospital of Siena, University of Siena, Italy.

Acquired factor XIII (FXIII) deficiency is an extremely rare and potentially fatal bleeding disorder. Immune-mediated FXIII deficiency is due to the development of anti-FXIII autoantibodies which may develop with concomitant conditions that cause immune dysregulation such as malignancies or autoimmune disorders. Clinical presentation includes delayed post-operative bleeding or spontaneous soft tissue hematomas and/or cerebral bleeding. Since screening coagulation laboratory tests (prothrombin time, activated partial thromboplastin time, and fibrinogen) are typically normal, acquired FXIII deficiency is likely to be overlooked and underdiagnosed. The management of immune-mediated FXIII deficiency is based on hemostatic therapy, autoantibody removal and eradication of the underlying etiology; however, no treatment guidelines are still available. Here we report a case of acquired FXIII deficiency associated with plasmablastic lymphoma, in order to raise awareness of this rare bleeding disorder and consent prompt life-saving management.

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How to Cite

Sammartano, V., Santoni, A., Zappone, E., Calzoni, P., Fineschi, D., Franceschini, E., … Puccetti, L. (2023). A case of acquired factor XIII deficiency secondary to plasmablastic lymphoma. Bleeding, Thrombosis and Vascular Biology, 2(1). https://doi.org/10.4081/btvb.2023.53
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