Complete remission achieved by steroid pulse therapy following rituximab treatment in a case with autoimmune haemorrhaphilia due to anti-factor XIII antibodies

Ogawa, Yoshiyuki; Mihara, Masahiro; Souri, Masayoshi; Yanagisawa, Kunio; Hayashi, Toshimasa; Kobayashi, Nobuhiko; Shimizu, Hiroaki; Iriuchishima, Hirono; Ishizaki, Takuma; Handa, Hiroshi; Osaki, Tsukasa; Nojima, Yoshihisa; Ichinose, Akitada

doi:10.1160/th14-04-0323

Cited by 5 publications

(10 citation statements)

References 13 publications

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“…Figure (A) shows such a clearance study demonstrating the considerably shortened half‐life of FXIII as a result of an anti‐FXIII‐B autoantibody . As opposed to the normal half‐life of FXIII in the circulation (9–12 days ), the half‐life of administered plasma FXIII concentrate was shortened to 17 h. Such pharmacokinetic monitoring of administered FXIII has also been reported in two Japanese studies . It is to be noted that in the case of anti‐FXIII‐A antibody with combined effect, because of the prompt inhibition of FXIII, activity measurement cannot be used for half‐life determination.…”

Section: Diagnosis and Classification Of Anti‐fxiii Antibodiesmentioning

confidence: 58%

“…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%

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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: 58%

Section: Diagnosis and Classification Of Anti‐fxiii Antibodiesmentioning

confidence: 99%

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

Muszbek

Pénzes

Katona

2018

Journal of Thrombosis and Haemostasis

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“…Neither CPA nor CyA were effective. The efficacy of rituximab for acquired inhibitors of coagulation factors, including AHXIII, has been reported in several studies (9,10). However, we were not able to use rituximab in this case because its use for this disorder is not currently accepted at our hospital.…”

Section: Discussionmentioning

confidence: 80%

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

et al. 2015

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A 65-year-old woman was admitted with acute intramuscular hemorrhage of the left gluteus medius and piriformis muscles and associated anemia. Blood tests showed low plasma factor XIII (FXIII) antigen and activity. A cross-mixing test revealed a concave "inhibitor" pattern and anti-FXIII-A subunit antibody was detected. The patient was diagnosed with autoimmune hemorrhaphilia resulting from anti-FXIII antibody. The bleeding has not recurred since the initiation of treatment with oral immunosuppressive agents. Although hemorrhagic acquired FXIII deficiency is a rare disorder, prompt recognition of the underlying mechanism can save lives.

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“…Unless treated promptly and aggressively [27], patient may die of haemorrhage either during the acute stage [28] or after a prolonged period. AH13 is a chronic intractable disease.…”

Section: Resultsmentioning

confidence: 99%

“…At the same time, AH13 is definitely a life-threatening disease. Unless treated promptly and aggressively [27], patient may die of haemorrhage either during the acute stage [28] or after a prolonged period. Finally, the prompt diagnosis and treatment of AH13 are essential to save patients' lives.…”

Section: Resultsmentioning

confidence: 99%

Clinical features of 32 new Japanese cases with autoimmune haemorrha‐philia due to anti‐factor XIII antibodies

2015

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Complete remission achieved by steroid pulse therapy following rituximab treatment in a case with autoimmune haemorrhaphilia due to anti-factor XIII antibodies

Cited by 5 publications

References 13 publications

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

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

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

Clinical features of 32 new Japanese cases with autoimmune haemorrha‐philia due to anti‐factor XIII antibodies

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