Anti-factor VIII Inhibitors and Lupus Anticoagulants in Haemophilia A Patients

Blanco, Alicia N.; Cardozo, Mauricio; Candela, M.; Santarelli, María Teresa; Bianco, R.; Lazzari, M.A.

doi:10.1055/s-0038-1656029

Cited by 32 publications

(32 citation statements)

References 22 publications

(32 reference statements)

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“…The prevalence of positive patients as detected by LA tests varied both between and within tests (see the different behaviors of the 2 dRVVT tests). The observed prevalence (27%) compares favorably with that in another series (5 ). However, for the vast majority of hemophiliacs in our series, the positivity was weak (see Table 1).…”

Section: Technical Briefssupporting

confidence: 78%

“…Previously published online at DOI: 10 Severe acute respiratory syndrome (SARS), caused by a novel coronavirus (SARS-CoV) (1)(2)(3)(4)(5), has affected 8096 people and produced 774 deaths in 29 countries/regions (6 ). The vital step in preventing and controlling future epidemics is to block transmission of infection through an effective quarantine policy, which in turn hinges on early diagnosis and confirmation of the disease, particularly by laboratory tests (7 ).…”

mentioning

confidence: 99%

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Lupus Anticoagulants and Their Relationship with the Inhibitors against Coagulation Factor VIII: Considerations on the Differentiation between the 2 Circulating Anticoagulants

et al. 2005

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Section: Technical Briefssupporting

confidence: 78%

mentioning

confidence: 99%

Lupus Anticoagulants and Their Relationship with the Inhibitors against Coagulation Factor VIII: Considerations on the Differentiation between the 2 Circulating Anticoagulants

et al. 2005

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“…They are not related to hemorrhage unless they are combined with thrombocytopenia or prothrombin deficiency [58]. Coexistence of LA and anti-FVIII antibodies has been reported by several authors [59,60]. Difficulties in determining the coexistence of anti-FVIII and LA with the currently established methods are also evident in most papers [61][62][63].…”

Section: Coexistence Of Lupus Anticoagulants and Fviii Inhibitorsmentioning

confidence: 99%

Immune Response to FVIII in Hemophilia A: An Overview of Risk Factors

Ghosh

Shetty

2009

Clinic Rev Allerg Immunol

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Development of inhibitors is perhaps the most serious complication of factor VIII (FVIII) replacement therapy, which can practically preclude efficient clinical management of patients with hemophilia A. Much effort therefore has been focused both in improving our understanding of the reasons for the formation of FVIII antibodies and to find alternative methods of treatment. Several patient-related factors have been related to the risk of inhibitor development such as ethnicity, FVIII gene mutation type, family history of inhibitors, HLA haplotype, polymorphisms in the promoter regions of IL 10 gene, single nucleotide polymorphisms of tumor necrosis factor alpha gene, and so on. In addition to the genetic determinants, there are several nongenetic factors which mainly include treatment characteristics like the type and purity of coagulation factor concentrates used for treatment, age at the time of initial treatment, initial doses of concentrate, mode of infusion, surgery, frequency of dosing prior to inhibitor development, and intensity of treatment or regular prophylaxis. Inflammatory processes in early childhood are under discussion as being an environmental factor that may modify the immune response to a foreign antigen. The genetic risks cannot be changed, while environmental factors may increase or decrease the inhibitor risk in an individual patient. In addition, there are other causes of inhibitor development against FVIII like stress, age, malignancy, infection, pregnancy, antibiotics, etc. Development of inhibitors in such cases happens in individuals who are not hemophilic and have normal plasma FVIII levels. Acquired inhibitors to FVIII in nonhemophiliacs (autoantibodies) pose a further challenge to treatment, as this is often associated with significant morbidity and mortality. Prognosis in case of autoantibodies is related to the underlying disease process and is associated with high mortality. Improved understanding of these complex interactions may lead to the development of preventive measures to minimize FVIII inhibitor formation. The modifiable risk factors for inhibitor formation may provide the key to predict and perhaps prevent the formation of inhibitors in hemophilia patients.

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“…The accurate diagnosis of FVIII inhibitors, in case of alloantibodies or auto-antibodies is complicated, mainly as patients could have a heterogeneous mix of antibodies, lupus anticoagulants or other non-specific antibodies [11][12][13][14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%

“…LA and FVIII inhibitors both prolong phospholipid-dependent coagulation tests, but differ with respect to target and clinical manifestation. In case of LA, addition of exogenous phospholipids will correct the prolonged APTT [12][13][14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%