By Pier Luigi Meroni
This publication, a part of the sequence infrequent illnesses of the Immune method, bargains accomplished, up to date assurance of the pathophysiology and administration of the antiphospholipid syndrome (APS). Immunologic and genetic points are mentioned and the pathogenic mechanisms accountable 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 awareness is paid to the character of the involvement of assorted organs or organ platforms in APS. particular chapters describe the therapy of different indicators, remedies of price in keeping off recurrences, and cutting edge remedy methods. The authors are senior specialists within the box who're aided by way of more youthful fellows, making sure that the publication can be educationally orientated. this useful quantity could be a helpful instrument for postgraduates in education and execs wishing to increase their wisdom of this particular syndrome.
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Extra resources for Antiphospholipid Antibody Syndrome: From Bench to Bedside
Conversely, 69, 50, and 53 out of 70 were able to rule out LAC when examining normal, heparinized, and deficient plasma (specificity, 99, 71, and 76 %). This survey showed that sensitivity is satisfactory, whereas specificity should be improved. 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.
Thromb Haemost 74:1185–1190 Thiagarajan P, Pengo V, Shapiro SS (1986) The use of the dilute Russell viper venom time for the diagnosis of lupus anticoagulants. Blood 68:869–874 Pengo V, Biasiolo A, Rampazzo P, Brocco T (1999) dRVVT is more sensitive than KCT or TTI for detecting lupus anticoagulant activity of anti-beta2-glycoprotein I autoantibodies. Thromb Haemost 81:256–258 Lawrie AS, Purdy G, Mackie IJ, Machin SJ (1997) Monitoring of oral anticoagulant therapy in lupus anticoagulant positive patients with the anti-phospholipid syndrome.
Beta2GPI is a plasma protein that may act as a scavenger molecule for lipopolysaccharide (LPS) through its C-terminal 3 Antiphospholipid Antibody Mechanisms of Thrombosis 27 part . Circulating plasma β2GPI exists in a circular form that, upon binding to suitable anionic surfaces as CL and other PL or to LPS, opens up to a J-shaped fishhook configuration . Such a conformational change exposes DI that has been suggested to be the main antigenic target for anti-β2GPI antibodies [12, 13]. In particular, it has been recently demonstrated that the antibodies recognize the sequence of arginine 39-arginine 43, aspartic acid 8-aspartic acid 9, and possibly the interlinker region between DI and DII, with R39 being the most important residue [14, 15].
Antiphospholipid Antibody Syndrome: From Bench to Bedside by Pier Luigi Meroni