Pathological coagulation parameters in as many as 54 patients with autoimmune acquired factor XIII deficiency due to anti-factor XIII autoantibodies.
autoantibody
coagulation factor deficiency
coagulation factor inhibitor
factor XIII
haemorrhagic diathesis
Journal
Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916
Informations de publication
Date de publication:
May 2021
May 2021
Historique:
revised:
04
03
2021
received:
26
01
2021
accepted:
06
03
2021
pubmed:
14
4
2021
medline:
21
9
2021
entrez:
13
4
2021
Statut:
ppublish
Résumé
Autoimmune factor XIII (FXIII) deficiency (AiF13D) due to anti-FXIII autoantibodies is an extremely rare, life-threatening bleeding disorder that mostly occurs in the elderly. The number of patients diagnosed with AiF13D has been increasing in Japan, probably because of the nationwide survey on AiF13D supported by the Japanese Ministry of Health, Labour and Welfare. To explore the pathologic characteristics of coagulation parameters in AiF13D. AiF13D-suspected cases were consulted, and underwent unified/integrated coagulation screening and were definitively diagnosed as AiF13D separately. AiF13D patients had lower FXIII antigen levels than non-AiF13D patients, but their values overlapped. Among a series of 22-item screening tests and their resulting parameters, the 'FXIII inhibitory potential' yielded by a 1:1 mixing test of the patient's and healthy control's plasma and its 'residual FXIII activity' in 54 AiF13D cases were most distinguishable from 139 non-AiF13D cases, followed by FXIII activity per se and FXIII-specific activity. While the cross-linked α AiF13D appears to induce a hypocoagulopathy combined with a hyper-fibrinolytic state secondary to severe FXIII deficiency caused by anti-FXIII autoantibodies, and the consequent bleeding further modifies its pathological conditions. In addition, the 1:1 mixing test of FXIII activity was confirmed to be a reliable screening method for AiF13D, especially when its derivative parameter, such as the 'FXIII inhibitory potential' or 'FXIII inhibitory potential ratio', is employed.
Substances chimiques
Autoantibodies
0
Factor XIII
9013-56-3
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
454-462Subventions
Organisme : Japanese Ministry of Health, Labor, and Welfare (MHLW)
Organisme : Japanese Ministry of Education, Culture, Sports, Science and Technology (MEXT)
Informations de copyright
© 2021 John Wiley & Sons Ltd.
Références
Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L. Factor XIII and fibrinogen SSC subcommittee of the ISTH. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011;9:1404-1406.
Muszbek L, Bereczky Z, Bagoly Z, Komáromi I, Katona É. Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev. 2011;91:931-972.
Ichinose A. Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control. Int J Hematol. 2012;95:362-370.
Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia. 2008;14:1190-1200.
Biswas A, Ivaskevicius V, Thomas A, Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie. 2014;34:160-166.
Egbring R, Kröniger A, Seitz R. Factor XIII deficiency: pathogenic mechanisms and clinical significance. Semin Thromb Hemost. 1996;22:419-425.
Ichinose A. Hemorrhagic acquired factor XIII (13) deficiency and acquired hemorrhaphilia 13 revisited. Semin Thromb Hemost. 2011;37:382-388.
Lorand L. Acquired inhibitors of fibrin stabilization: a class of hemorrhagic disorders of diverse origins. In: Green D, ed. Anticoagulants Physiologic, Pathologic, and Pharmacologic. CRC Press; 1994:169-191.
Egbring R, Kröniger A, Seitz R. Erworbene inhibitoren gegen Faktor XIII. Ham ostaseologie. 1996;16:174-179.
Ichinose A, Japanese Collaborative Research Group (JCRG) on AH13 Hemorrhagic Acquired Coagulopathies. Inhibitors of factor XIII/13 in older patients. Semin Thromb Hemost. 2014;40:704-711.
Ichinose A, Souri M, Japanese collaborative research group on "Acquired haemorrha-philia due to factor XIII deficiency". As many as 12 cases with haemorrhagic acquired factor XIII deficiency due to its inhibitors were recently found in Japan. Thromb Haemost. 2011;105:925-927.
Ichinose A, Osaki T, Souri M, Japanese Collaborative Research Group (JCRG) on AH13 (supported by the Japanese Ministry of Health, Labor, and Welfare). Clinical features of 32 new Japanese cases with autoimmune hemorrha-philia due to anti-factor XIII antibodies. Haemophilia. 2015;21:653-658.
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.
Franchini M, Frattini F, Crestani S, Bonfanti C. Acquired FXIII inhibitors: a systematic review. J Thromb Thrombolysis. 2013;36:109-114.
Boehlen F, Casini A, Chizzolini C, et al. Acquired factor XIII deficiency: a therapeutic challenge. Thromb Haemost. 2013;109:479-487.
Kessel R, Hu C, Shore-Lesserson L, Rand J, Manwani D. A child with acquired factor XIII deficiency: case report and literature review. Haemophilia. 2013;19:814-826.
Tone KJ, James TE, Fergusson DA, 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.
Karimi M, Peyvandi F, Naderi M, Shapiro A. Factor XIII deficiency diagnosis: challenges and tools. Int J Lab Hematol. 2018;40:3-11.
Beckman JD, Kasthuri RS, Wolberg AS, Ma AD. Challenges in diagnosis and management of acquired factor XIII (FXIII) inhibitors. Haemophilia. 2018;24:e417-e420.
Sugiyama H, Uesugi H, Suzuki S, Tanaka K, Souri M, Ichinose A. Aggressive fatal case of autoimmune hemorrhaphilia resulting from anti-factor XIII antibodies. Blood Coagul Fibrinolysis. 2013;24:85-89.
Rodeghiero F, Tosetto A, Abshire T, et al. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost. 2010;8:2063-2065.
Schulman S, Kearon C, Subcommittee on Control of Anticoagulation of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Definition of major bleeding in clinical investigations of antihemostatic medicinal products in non-surgical patients. J Thromb Haemost. 2005;3:692-694.
Baudo F, Collins P, Huth-Kühne A, et al. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood. 2012;120:39-46.
Ichinose A. Immune-mediated acquired coagulation factor deficiencies: state-of-the-art in diagnosis and management. Rinsho Ketsueki. 2019;60:667-679.
Souri M, Osaki T, Ichinose A. Anti-factor XIII A subunit (FXIII-A) autoantibodies block FXIII-A2B2 assembly and steal FXIII-A from native FXIII-A2B2. J Thromb Haemost. 2015;13:802-814.
Osaki T, Sugiyama D, Magari Y, Souri M, Ichinose A. Rapid immunochromatographic test for detection of anti-factor XIII A subunit antibodies can diagnose 90 % of cases with autoimmune haemorrhaphilia XIII/13. Thromb Haemost. 2015;113:1347-1356.
Ichinose A. Diagnosis and treatment of acquired factor XIII/13 deficiencies: for all doctors treating the MHLW's designated intractable diseases. Rinsho Ketsueki. 2015;56:2110-2122.
Ichinose A, Kohler HP, Philippou H, Factor XIII and Fibrinogen SSC Subcommittee of the ISTH. Recommendation for ISTH/SSC criterion for autoimmune acquired factor XIII/13 deficiency. Thromb Haemost. 2015;2016(116):772-774.
Tsuda M, Kiyasu J, Sugio K, et al. Spontaneous splenic rupture accompanied by hepatic arterial dissection in a patient with autoimmune haemorrhaphilia due to anti-factor XIII antibodies. Haemophilia. 2016;22(4):e314-e317.
Ichinose A, Souri M. Reduced difference of α₂-plasmin inhibitor levels between plasma and serum in patients with severe factor XIII deficiency, including autoimmune hemorrhaphilia due to anti-factor XIII antibodies. Int J Hematol. 2012;95:47-50.
Kotake T, Souri M, Takada K, Kosugi S, Nakata S, Ichinose A. Report of a patient with chronic intractable autoimmune hemorrhaphilia due to anti-Factor XIII/13 antibodies who died of hemorrhage after sustained clinical remission for three years. Int J Hematol. 2015;101:598-602.
Ajzner E, Schlammadinger A, Kerényi A, 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.
Ogawa Y, Mihara M, Souri M, et al. Complete remission achieved by steroid pulse therapy following rituximab treatment in a case with autoimmune haemorrhaphilia due to anti-factor XIII antibodies. Thromb Haemos. 2014;112:831-833.
Fraser SR, Booth NA, Mutch NJ. The antifibrinolytic function of factor XIII is exclusively expressed through α₂-antiplasmin cross-linking. Blood. 2011;117:6371-6374.
van Aken PJ, Emeis JJ. Organization of experimentally induced arterial thrombosis in rats: the first six days. Artery. 1982;11:156-173.
Wohner N. Role of cellular elements in thrombus formation and dissolution. Cardiovasc Hematol Agents Med Chem. 2008;6:224-228.
Plow EF. Leukocyte elastase release during blood coagulation. A potential mechanism for activation of the alternative fibrinolytic pathway. J Clin Invest. 1982;69:564-572.
Machovich R, Owen WG. The elastase-mediated pathway of fibrinolysis. Blood Coagul Fibrinolysis. 1990;1:79-90.
Rondaij MG, Bierings R, Kragt A, van Mourik JA, Voorberg J. Dynamics and plasticity of Weibel-Palade bodies in endothelial cells. Arterioscler Thromb Vasc Biol. 2006;26:1002-1007.
Lenting PJ, Christophe OD, Denis CV. von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015;125:2019-2028.