Epitope analysis of human monoclonal antibodies from a patient with autoimmune factor <scp>XIII</scp> deficiency reveals their inhibitory mechanisms

Osaki, Tsukasa; Souri, Masayoshi; Ozawa, Tatsuhiko; Muraguchi, Atsushi; Ichinose, Akitada

doi:10.1002/1873-3468.14606

Cited by 2 publications

(1 citation statement)

References 31 publications

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“…Since autoantibodies are generally polyclones, 61,62 these three types are present in varying proportions in a single patient plasma; some may be predominant, or the proportion may change over time, requiring physicians' attention. In blood, coagulation factor-bound (complexed) and -unbound (free) autoantibodies exist.…”

Section: Concept Of Anticoagulation Factor Autoantibodiesmentioning

confidence: 99%

Diagnosis and Treatment of Autoimmune Acquired Coagulation Factor Deficiencies: An Evidence-Based Review of Japanese Practice

Ichinose,

Osaki,

Souri

2024

Semin Thromb Hemost

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Among the acquired coagulation factor deficiencies, autoimmune coagulation factor deficiencies (AiCFD) are rare and result from autoantibody production against coagulation factors. In Japan, a nationwide survey on AiCFD has been conducted since 2009. Autoimmune factor XIII, factor VIII, von Willebrand factor, factor V, and factor X deficiencies (AiF13D, AiF8D, AiVWFD, AiF5D, and AiF10D, respectively) have been enacted as “designated intractable disease-282.” The incidence of AiF8D, AiF13D, and AiF5D was 1.83, 0.044, and 0.038 per million people/year, respectively, whereas that of AiVWFD and AiF10D was not calculable owing to the small number of patients. AiF13D and AiF8D were often idiopathic, whereas AiVWFD was often associated with plasma cell neoplasms. Epistaxis was a characteristic symptom of AiVWFD, intramuscular bleeding was frequent in AiF13D and AiF8D, and subcutaneous bleeding (purpura) was frequent in AiF13D and AiF10D, although none were specific to any one disease. Differential diagnosis cannot be made based on bleeding symptoms alone; therefore, rapid and accurate testing is mandatory. Definitive diagnosis of AiCFD necessitates identifying the presence of coagulation factor “inhibitors” and/or “autoantibodies.” Therefore, these tests should be performed upon unexplained severe acquired coagulation factor deficiencies. The mainstay of treatment for AiCFD was hemostatic therapy and autoantibody eradication therapy, which included the replacement of coagulation factors or “bypass” agents and administration of immunosuppressants. The rate of hemorrhagic death was high in AiF13D (13%), followed by AiF5D (7%) and Ai10D (5%); therefore, early diagnosis and optimal treatment are essential for AiCFDs. Given the unknown long-term prognosis, “intractable disease platform registries” have begun to accumulate in Japan.

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Section: Concept Of Anticoagulation Factor Autoantibodiesmentioning

confidence: 99%