https://doi.org/10.4081/btvb.2023.53
A case of acquired factor XIII deficiency secondary to plasmablastic lymphoma
Submitted: 25 October 2022
Accepted: 20 February 2023
Published: 9 March 2023
Accepted: 20 February 2023
Abstract Views: 516
PDF: 317
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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.
Yan MTS, Rydz N, Goodyear D, Sholzberg M. Acquired factor XIII deficiency: A review. Transfus Apher Sci 2018;57:724-30.
DOI: https://doi.org/10.1016/j.transci.2018.10.013
Chou SC, Lin CY, Yen CT, et al. Acquired FXIII inhibitor: Patient characteristics and treatment outcome, a case series in Taiwan. J Formos Med Assoc 2021;120:411-4.
DOI: https://doi.org/10.1016/j.jfma.2020.05.032
Dorgalaleh A, Rashidpanah J. Blood coagulation factor XIII and factor XIII deficiency. Blood Rev 2016;30:461-75.
DOI: https://doi.org/10.1016/j.blre.2016.06.002
Muszbek L, Katona É. Diagnosis and Management of Congenital and Acquired FXIII Deficiencies. Semin Thromb Hemost 2016;42:429-39.
DOI: https://doi.org/10.1055/s-0036-1572326
Ichinose A, Kohler HP, Philippou H. Factor XIII and Fibrinogen SSC Subcommittee of the ISTH. Recommendation for ISTH/SSC Criterion 2015 for autoimmune acquired factor XIII/13 deficiency. Thromb Haemost 2016;116:772-4.
DOI: https://doi.org/10.1160/TH16-05-0362
Durda MA, Wolberg AS, Kerlin BA. State of the art in factor XIII laboratory assessment. Transfus Apher Sci 2018;57:700-4.
DOI: https://doi.org/10.1016/j.transci.2018.07.006
Ichinose A. Japanese Collaborative Research Group on AH13. Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients. Blood Rev 2017;31:37-45.
DOI: https://doi.org/10.1016/j.blre.2016.08.002
Suarez-Londono JA, Rohatgi A, Antoine-Pepeljugoski C, Braunstein MJ. Aggressive presentation of plasmablastic myeloma. BMJ Case Rep 2020;13:e234436.
DOI: https://doi.org/10.1136/bcr-2020-234436
Ferretti A, Baldacci E, Fazio F, et al. Acquired FXIII deficiency and AL amyloidosis: A case of a rare association. Transfus Apher Sci 2020;59:102903.
DOI: https://doi.org/10.1016/j.transci.2020.102903
Kariyawasan CC, Hughes DA, Jayatillake MM, Mehta AB. Multiple myeloma: causes and consequences of delay in diagnosis. QJM 2007;100:635-40.
DOI: https://doi.org/10.1093/qjmed/hcm077
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
Copyright (c) 2023 The Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Most read articles by the same author(s)
- Luca Puccetti, Vincenzo Sammartano, Federico Caroni, Margherita Malchiodi, Paola Calzoni, Eleonora Franceschini, Lucrezia Galasso, Monica Bocchia, Safety of COVID-19 mRNA vaccination in patients with history of acquired hemophilia A: a case series , Bleeding, Thrombosis and Vascular Biology: Vol. 1 No. 3 (2022)
Similar Articles
- Juan Eirís, Marina Suárez-Terrón, Pablo Granados, David Martínez-Campuzano, Ana Rosa Cid, Saturnino Haya, Santiago Bonanad, Allopurinol-induced acquired von Willebrand syndrome , Bleeding, Thrombosis and Vascular Biology: Vol. 2 No. 4 (2023)
- Daniela Poli, Emilia Antonucci, Gualtiero Palareti, Roberto Facchinetti, Pietro Falco, Giuseppina Serricchio, Teresa Lerede, Lucilla Masciocco, Paolo Gresele, Sophie Testa, Major bleedings in mechanical prosthetic heart valves patients on Vitamin K antagonist treatment. Data from the PLECTRUM Study , Bleeding, Thrombosis and Vascular Biology: Vol. 1 No. 2 (2022)
- Domenico Prisco, Irene Mattioli, Raffaele De Caterina, Alessandra Bettiol, The new era of anticoagulation: factor XI and XII inhibitors , Bleeding, Thrombosis and Vascular Biology: Vol. 2 No. 2 (2023)
- Mattia Galli, C. Michael Gibson, Dominick J. Angiolillo, Factor XI inhibitors in adjunct to antiplatelet therapy: the ultimate dual-pathway inhibition? , Bleeding, Thrombosis and Vascular Biology: Vol. 2 No. 3 (2023)
- Samantha Pasca, Cristina Santoro, Chiara Ambaglio, Marisanta Napolitano, Marta Milan, Letizia Natali, Silvia Nannizzi, Filippo Mori, Paolo Simioni, Ezio Zanon, Comparison among three different bleeding scores and the thrombin generation assay to assess the different hemorrhagic phenotypes in patients with FVII deficiency , Bleeding, Thrombosis and Vascular Biology: Vol. 1 No. 2 (2022)
- Roberto Mario Santi, Annamaria Fulghesu, Ezio Zanon, Erica De Candia, Elvira Grandone, Giancarlo Di Renzo, Claudia Succu, Valentina Tosto, Vincenzina Bruni, Paolo Gresele, Diagnosis and management of abnormal uterine bleeding in adolescence , Bleeding, Thrombosis and Vascular Biology: Vol. 3 No. 1 (2024)
- Giovanni de Gaetano, Da la scheggia rotta usciva insieme parole e sangue: an unusual case of bleeding and the metaphor of a clinical trial , Bleeding, Thrombosis and Vascular Biology: Vol. 3 No. 3 (2024)
- Luca Puccetti, Vincenzo Sammartano, Federico Caroni, Margherita Malchiodi, Paola Calzoni, Eleonora Franceschini, Lucrezia Galasso, Monica Bocchia, Safety of COVID-19 mRNA vaccination in patients with history of acquired hemophilia A: a case series , Bleeding, Thrombosis and Vascular Biology: Vol. 1 No. 3 (2022)
- Maria Barca-Hernando, Victor Garcia-Garcia, Luis Jara-Palomares, Location of metastasis and complications in patients with venous thromboembolism and cancer: systematic review , Bleeding, Thrombosis and Vascular Biology: Vol. 3 No. s1 (2024): 12th ICTHIC
- Marcello Di Nisio, Matteo Candeloro, Nicola Potere, Ettore Porreca, Jeffrey I. Weitz, Factor XI inhibitors: a new option for the prevention and treatment of cancer-associated thrombosis , Bleeding, Thrombosis and Vascular Biology: Vol. 3 No. s1 (2024): 12th ICTHIC
You may also start an advanced similarity search for this article.
