An Acquired Inhibitor of Factor XIII with a Qualitative Abnormality of Fibrin Cross-Linking

Fear, Janine; Miloszewski, K.; Losowsky, M. S.

doi:10.1159/000206606

Cited by 17 publications

(9 citation statements)

References 8 publications

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“…Nearly 25% (8/34) of patients died secondary to haemorrhage, with three due to cerebral haemorrhage. One patient died due to cerebral haemorrhage despite improved posttreatment FXIII level .…”

Section: Prognosismentioning

confidence: 99%

A child with acquired factor XIII deficiency: case report and literature review

Kessel

Shore‐Lesserson

et al. 2013

Haemophilia

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Factor XIII (FXIII) deficiency is a rare bleeding disorder, which can result in life threatening hemorrhage. Rarer still is acquired FXIII deficiency, in which the disorder is due to autoantibodies that inhibit the factor. To describe one of the youngest reported patients with this condition. To discuss the challenges we encountered in monitoring response with the available assays. To review the literature and provide a review of all acquired FXIII cases. We present the case of our patient, a 9-year-old girl with acquired FXIII deficiency. We present a comprehensive review of all acquired FXIII deficiency cases reported globally in English, with focus on clinical presentation, diagnostic assays, treatment and prognosis. There is no current standard for therapy and measuring response to therapy can be complicated by limitations of assays in the presence of inhibitors. Clinicians should be aware of acquired FXIII deficiency as a potentially life threatening bleeding disorder even in young children. The case presented illustrates a young patient with acquired FXIII deficiency with a good clinical response to cryoprecipitate and difficulty in hemostasis monitoring utilizing clinically available assays.

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Section: Prognosismentioning

confidence: 99%

A child with acquired factor XIII deficiency: case report and literature review

Kessel

Shore‐Lesserson

et al. 2013

Haemophilia

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“…Almost all patients with inherited FXIII deficiency have zero FXIII activity if untreated and will be picked up by this test. If clot solubility in these reagents is found, it is important to perform some simple mixing experiments with normal plasma to make sure that the observed clot solubility is the result of FXIII deficiency and not due to the presence of a FXIII inhibitor ( Fear et al , 1984 ). If the diagnosis of FXIII deficiency is suggested by the solubility test, it should be confirmed by estimation of FXIII activity using one of several quantitative, amine–casein incorporation assays, e.g.…”

Section: Diagnosis Of Fxiii Deficiencymentioning

confidence: 99%

Factor Xiii Deficiency

1999

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“…If urea lysis tests bring positive results and FXIII inhibitors are suspected to be present, you can have a correcting test of urea lysis: mix the plasma of a normal person and that of a patient in different proportions. The dissolving time of clots which form in mixed plasma in 5 mol/l urea solution will not be corrected to the normal level [8].…”

Section: Discussion and Review Of Literaturementioning

confidence: 99%

Acquired coagulation factor XIII deficiency

Jia

Bin

2016

Blood Coagulation &Amp; Fibrinolysis

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The main objective of the study is to summarize the clinical characteristics of acquired factor XIII (FXIII) deficiency caused by a spontaneous FXIII inhibitor. Here we report a new case of acquired FXIII deficiency caused by FXIII inhibitor and review the medical literature regarding the characteristics and treatment of this disorder. FXIII deficiency caused by FXIII inhibitors is rare and of uncertain pathogenesis. Experience with therapeutic measures is limited to data from case reports. Immunosuppressive drugs may reduce autoantibodies or inhibit the cell clone generating the antibodies and may have been of benefit in our patient. The impact of such therapy on patient prognosis is incompletely known.

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An Acquired Inhibitor of Factor XIII with a Qualitative Abnormality of Fibrin Cross-Linking

Cited by 17 publications

References 8 publications

A child with acquired factor XIII deficiency: case report and literature review

A child with acquired factor XIII deficiency: case report and literature review

Factor Xiii Deficiency

Acquired coagulation factor XIII deficiency

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