Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure

Rabik, Cara A; Atkinson, Meredith A.; Sule, Sangeeta; Strouse, John J.

doi:10.1111/trf.14185

Cited by 3 publications

(2 citation statements)

References 19 publications

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“…190 Although much less common, hypocoagulation due to inhibitors of factors or antibodymediated thrombocytopenia is described in these disease states. 191 Table 6 presents TEG/ROTEM variables associated with various systemic autoimmune diseases and the potential implications of those variables. Studies have revealed that patients with active systemic lupus erythematosus and related disorders show a distinct hematologic pattern characteristic of a hypercoagulable state; however, there are varying degrees of changes in VET variables in patients with active disease, which suggests diversity in associated pathophysiologic mechanisms.…”

Section: Systemic Autoimmune Diseasesmentioning

confidence: 99%

“…Another study attributed the hypercoagulability observed in lupus‐related disorders to increased PAI‐1 levels, PLT activation, and neutrophil extracellular traps 190 . Although much less common, hypocoagulation due to inhibitors of factors or antibody‐mediated thrombocytopenia is described in these disease states 191 …”

Section: Benign Hematologic Disordersmentioning

confidence: 99%

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Viscoelastic testing in benign hematologic disorders: Clinical perspectives and future implications of point‐of‐care testing to assess hemostatic competence

et al. 2020

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Viscoelastic tests (VETs) have been used routinely for liver transplantation, cardiac surgery, and trauma, but only recently have found clinical utility in benign hematologic disorders. Therefore, guidelines for diagnosis and treatment of these disorders based on viscoelastic variables have been adapted from the existing transplant, cardiothoracic surgery, and trauma resuscitation literature. As a result, diagnostic and therapeutic strategies for benign hematologic disorders utilizing VETs are not uniform. Accordingly, even though there has been a recent increase in the utilization of VET for the diagnosis and treatment of such disorders, the literature is still in its early stages. Analysis of point‐of‐care viscoelastic tracings from benign hematologic disorders has the potential to allow prompt recognition of disease and to guide patient‐specific intervention. Here we present a review describing the application of VETs to benign hematologic disorders.

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Section: Systemic Autoimmune Diseasesmentioning

confidence: 99%

Section: Benign Hematologic Disordersmentioning

confidence: 99%

Viscoelastic testing in benign hematologic disorders: Clinical perspectives and future implications of point‐of‐care testing to assess hemostatic competence

et al. 2020

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Ruptured hemorrhagic corpus luteum as a presenting symptom of systemic lupus erythematous

et al. 2020

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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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Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure

Abstract: Treatment with either plasma-derived FXIII or cryoprecipitate is an effective treatment to normalize the kTEG variables and clinical bleeding diatheses associated with acquired FXIII inhibitors. Higher doses may be needed in patients with high-titer inhibitor.

Cited by 3 publications

References 19 publications

Viscoelastic testing in benign hematologic disorders: Clinical perspectives and future implications of point‐of‐care testing to assess hemostatic competence

Viscoelastic testing in benign hematologic disorders: Clinical perspectives and future implications of point‐of‐care testing to assess hemostatic competence

Ruptured hemorrhagic corpus luteum as a presenting symptom of systemic lupus erythematous

Acquired factor XIII deficiency

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