By Pier Luigi Meroni
This booklet, a part of the sequence infrequent illnesses of the Immune procedure, bargains finished, up to date insurance of the pathophysiology and administration of the antiphospholipid syndrome (APS). Immunologic and genetic features are mentioned and the pathogenic mechanisms chargeable for such phenomena as APS-mediated thrombosis and being pregnant loss/complications are defined. the most scientific manifestations, class standards and diagnostic instruments are pointed out, and shut recognition is paid to the character of the involvement of assorted organs or organ structures in APS. particular chapters describe the therapy of the several indicators, remedies of worth in averting recurrences, and cutting edge therapy techniques. The authors are senior specialists within the box who're aided through more youthful fellows, making sure that the ebook is additionally educationally orientated. this useful quantity should be a important device for postgraduates in education and execs wishing to increase their wisdom of this particular syndrome.
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Additional info for Antiphospholipid Antibody Syndrome: From Bench to Bedside
We have recently demonstrated that LAC with associated aCL and aβ2GPI antibody positivity (triple positivity) is a risk factor for thromboembolic recurrence. Moreover, LAC in these patients constantly remains positive with time. Thromboembolic events may occur anywhere without provocation. 0). A first distinction in methods to detect LAC in relation to clinical manifestation of APS considered LAC to be clinically relevant when detected by means of dRVVT. In fact, the dRVVT rather than the KCT profile may better identify β2GPIdependent LAC and is associated with thrombosis in LAC-positive patients.
Cruz-Tapias P et al (2012) Infections and vaccines in the etiology of antiphospholipid syndrome. Curr Opin Rheumatol 24:389–393 10. Agar C et al (2011) Beta2-glycoprotein I: a novel component of innate immunity. Blood 117:6939–6947 11. De Laat B et al (2011) Autoantibodies directed against domain I of beta2-glycoprotein I. Curr Rheumatol Rep 13:70–76 12. Iverson GM et al (1998) Anti-β2 glycoprotein I (β2GPI) autoantibodies recognize an epitope on the first domain of β 2GPI. Proc Natl Acad Sci U S A 95:15542–15546 13.
APL procoagulant mechanism(s) has been initially related to the antibody’s binding to β2GPI or PT and the consequent interference with the natural anticoagulant 28 Fig. 1 Schematic representation of the coagulation cascade. L. Meroni et al. Extrinsic pathway VII Intrinsic pathway TF VIIa XII PK HK XI HK XIIa IX Ca2+ VIIa/TF XIa Ca2+ PL IXa VIIIa X VIII Xa Ca2+ PL PT PL Va Fibrinogen V TH Fibrin monomer Fibrin polymer XIIIa XIII Crosslinked fibrin polymer system of protein C and with the reduction of fibrinolysis.
Antiphospholipid Antibody Syndrome: From Bench to Bedside by Pier Luigi Meroni