Autoimmune Hemorrhaphilia Resulting from Autoantibody against the A Subunit of Factor XIII

Uchida, Emiko; Watanabe, Ken; Arai, Reina; Yamamoto, Masahide; Souri, Masayoshi; Osaki, Tsukasa; Ichinose, Akitada; Miura, Osamu; Koyama, Takatoshi

doi:10.2169/internalmedicine.54.4791

Cited by 3 publications

(3 citation statements)

References 14 publications

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“…In the assay the respective FXIII species are blotted to nitrocellulose membrane and the reaction with the patient's diluted plasma or purified IgG is detected by labeled secondary antibody. This technique was intensively used by the Japanese group, and contributed to the diagnosis of a relatively high number of patients with anti‐FXIII antibodies (see Table S1 and references ). In the latter cases rFXIII‐A 2 and rFXIII‐B 2 were used as antigen.…”

Section: Diagnosis and Classification Of Anti‐fxiii Antibodiesmentioning

confidence: 99%

“…In a few studies, changes in the BU titer were also used for monitoring the effectiveness of eradication therapy . Residual FXIII activity in a 1 : 1 mix of the patient's and normal plasma was also used to monitor the therapeutic effectiveness . In our experience the most precise information on the neutralizing capacity of an autoantibody is provided by IC50 determination.…”

Section: Analyses Of Patients With Anti‐fxiii Autoantibodiesmentioning

confidence: 99%

“…In a few cases the binding assays were also useful in identifying the FXIII subunit and the activation products of FXIII to which the anti‐FXIII antibody had considerable affinity. ELISA‐type and dot blot assays were used in eight and seven cases. Occasionally the techniques used for the characterization of antibody binding were western blotting , the immuno‐chromatographic test and the surface plasmon resonance (SPR) technique .…”

Section: Analyses Of Patients With Anti‐fxiii Autoantibodiesmentioning

confidence: 99%

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Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences

Muszbek

Pénzes

Katona

2018

Journal of Thrombosis and Haemostasis

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Acquired FXIII deficiencies caused by autoantibodies against FXIII subunits represent rare but very severe bleeding diatheses. Alloantibodies in FXIII-deficient patients also cause life-threatening bleeding complications, but they develop extremely rarely. In this review we provide an overview of the diagnosis and classification of anti-FXIII antibodies and analyze 48 patients with autoimmune FXIII deficiency and four additional FXIII-deficient patients who developed anti-FXIII alloantibody. The patients were collected from peer-reviewed publications from which relevant data could be extracted. With the exception of two cases the antibodies were directed against FXIII-A. The difficulties in the diagnosis of FXIII deficiency in the presence of anti-FXIII antibodies are discussed and a scheme for the functional classification of the anti-FXIII antibodies is recommended. The three main categories are neutralizing and non-neutralizing antibodies and antibodies with combined effect. The methods being used for detecting and quantifying the inhibitory effect on FXIII activation and on the transglutaminase activity of activated FXIII are summarized and techniques for the classification of neutralizing anti-FXIII antibodies are outlined. The importance of clearance studies in these cases is emphasized. Binding assays, useful for the identification of non-neutralizing and combined type antibodies, were collected from the literature and their informative power is demonstrated by examples. The most frequently occurring bleeding symptoms in patients with anti-FXIII antibodies were soft tissue bleeding; intracranial bleedings also occurred, but less frequently than in inherited FXIII deficiency. Treatment of such patients is extremely challenging; the main aim should be eradication of the antibody.

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Section: Diagnosis and Classification Of Anti‐fxiii Antibodiesmentioning

confidence: 99%

Section: Analyses Of Patients With Anti‐fxiii Autoantibodiesmentioning

confidence: 99%

Section: Analyses Of Patients With Anti‐fxiii Autoantibodiesmentioning

confidence: 99%

See 1 more Smart Citation

Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences

Muszbek

Pénzes

Katona

2018

Journal of Thrombosis and Haemostasis

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Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan

Souri,

Osaki,

Ichinose

2024

Int J Hematol

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Acquired factor XIII deficiency

Duranteau,

Tatar,

Demulder

et al. 2023

European Journal of Anaesthesiology Intensive Care

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Acquired factor XIII (FXIII) deficiency is a very rare haemostatic defect that can be either immune (rare development of an autoantibody targeting FXIII epitopes) or nonimmune (diminished synthesis or increased consumption of the same factor). The aim of this study is to review the symptomatology, the diagnostic method used, but above all to determine the most frequently used and potentially most effective treatment for acquired FXIII deficiency. PubMed, Medline, embase/Ovid databases were queried from 1 January 2012 to 3 April 2022. Data extraction was performed using the keywords ‘Acquired FXIII deficiency.’ The systematic search identified 474 records. After screening titles and abstracts, only 36 articles met the eligibility criteria. The mean age of all patients was 57.6 [range, 1–84] years. The male to female ratio was 35 : 25. The majority of cases described were due an autoimmune reaction with antibody production (24 manuscripts), only six manuscripts described consumption. The most prevalent symptoms were local haematoma (31). Six cases died, two from haemorrhagic shock, two from haemorrhagic stroke, one from respiratory distress, and 1 from septic shock. Given the patient outcomes, this review confirms that the most appropriate treatment consists of one of the following elements or a combination of several of these elements: FXIII concentrate, corticosteroids, cyclophosphamide, rituximab for autoimmune cases, and FXIII concentrate supplementation only in case of consumption.

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Autoimmune Hemorrhaphilia Resulting from Autoantibody against the A Subunit of Factor XIII

Cited by 3 publications

References 14 publications

Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences

Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences

Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan

Acquired factor XIII deficiency

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