Acquired factor XIII deficiency and its unprecedented association with multiple myeloma: case report and literature review.
Journal
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis
ISSN: 1473-5733
Titre abrégé: Blood Coagul Fibrinolysis
Pays: England
ID NLM: 9102551
Informations de publication
Date de publication:
01 Jul 2024
01 Jul 2024
Historique:
medline:
8
7
2024
pubmed:
8
7
2024
entrez:
8
7
2024
Statut:
ppublish
Résumé
This case involves a 58-year-old patient, with no personal or family history of bleeding, diagnosed with stage III-A IgG Kappa multiple myeloma in 2016 and underwent autografting in 2017. In March 2022, he suffered a myeloma relapse. In October 2022, the patient presented with a large spontaneous compressive hematoma in the left shoulder and hemithorax, requiring two surgical procedures. The platelet count and hemostasis parameters were within normal range. Further diagnostic testing revealed acquired factor XIII deficiency and acquired thrombopathy. Diagnostic challenges arose from the absence of specific assays for the B subunit of Factor XIII. Treatment involved transfusions and corticosteroids, but efficacity was limited. The patient's response to chemotherapy raised questions about the involvement of the monoclonal component. Subsequent follow-ups showed factor XIII levels fluctuating, even without symptoms. The patient was deemed ineligible for autograft due to a significant risk of hemorrhage.
Identifiants
pubmed: 38973520
doi: 10.1097/MBC.0000000000001311
pii: 00001721-202407000-00012
doi:
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
293-295Informations de copyright
Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.
Références
Ichinose A. Hemorrhagic acquired factor XIII (13) deficiency and acquired hemorrhaphilia 13 revisited. Semin Thromb Hemost 2011; 37:382–388.
Dorgalaleh A, Rashidpanah J. Blood coagulation factor XIII and factor XIII deficiency. Blood Rev 2016; 30:461–475.
Yan MTS, Rydz N, Goodyear D, Sholzberg M. Acquired factor XIII deficiency: a review. Transfus Apher Sci 2018; 57:724–730.
Ichinose A. Japanese Collaborative Research Group on AH13. Autoimmune acquired factor XIII deficiency due to antifactor XIII/13 antibodies: a summary of 93 patients. Blood Rev 2017; 31:37–45.
Ichinose A, Kohler HP, Philippou H, Factor XIII. Fibrinogen SSC Subcommittee of the ISTH. Recommendation for ISTH/SSC Criterion 2015 for autoimmune acquired factor XIII/13 deficiency. Thromb Haemost 2016; 116:772–774.
Luo Y, Zhang G, Zuo W, Zheng W, Dai C. Acquired factor XIII inhibitor in monoclonal gammopathy of undetermined significance: characterization and cross-linked fibrin ultrastructure. Ann Hematol 2010; 89:833–834.
Choufani EB, Sanchorawala V, Ernst T, Quillen K, Skinner M, Wright DG, Seldin DC. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood 2001; 97:1885–1887.
Furie B, Voo L, McAdam KP, Furie BC. Mechanism of factor X deficiency in systemic amyloidosis. N Engl J Med 1981; 304:827–830.
Muszbek L, Katona É. Diagnosis and management of congenital and acquired FXIII deficiencies. Semin Thromb Hemost 2016; 42:429–439.
Katona É, Pénzes K, Molnár É, Muszbek L. Measurement of factor XIII activity in plasma. Clin Chem Lab Med 2012; 50:1191–1202.
Souri M, Osaki T, Ichinose A. Antifactor XIII A subunit (FXIII-A) autoantibodies block FXIII-A2 B2 assembly and steal FXIII-A from native FXIII-A2 B2. J Thromb Haemost JTH 2015; 13:802–814.
Luo YY, Zhang GS. Acquired factor XIII inhibitor: clinical features, treatment, fibrin structure and epitope determination. Haemoph Off J World Fed Hemoph 2011; 17:393–398.
Ajzner E, Schlammadinger A, Kerényi A, Bereczky Z, Katona E, Haramura G, et al. Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency. Blood 2009; 113:723–725.
Tone KJ, James TE, Fergusson DA, Tinmouth A, Tay J, Avey MT, et al. Acquired factor XIII inhibitor in hospitalized and perioperative patients: a systematic review of case reports and case series. Transfus Med Rev 2016; 30:123–131.