By Pier Luigi Meroni
This ebook, a part of the sequence infrequent illnesses of the Immune process, bargains entire, updated insurance of the pathophysiology and administration of the antiphospholipid syndrome (APS). Immunologic and genetic elements 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, type standards and diagnostic instruments are pointed out, and shut consciousness is paid to the character of the involvement of assorted organs or organ structures in APS. particular chapters describe the therapy of different indicators, remedies of price in fending off recurrences, and leading edge therapy techniques. The authors are senior specialists within the box who're aided through more youthful fellows, making sure that the booklet can also be educationally orientated. this useful quantity might be a worthy software for postgraduates in education and pros wishing to increase their wisdom of this particular syndrome.
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Additional info for Antiphospholipid Antibody Syndrome: From Bench to Bedside
APL reactivity with platelets has been shown only after their pre-activation by agonists such as thrombin or collagen. The reason for this was thought to be related to the exposure of phosphatidylserine (PS) on the cell outer membrane after platelet activation and the consequent binding of the cationic β2GPI. However, β2GPI can also bind two surface receptors on platelets. Apolipoprotein E receptor 2’ (ApoER2’) is a member of low-density lipoprotein (LDL) receptor family expressed by platelets; its LDL-binding domain I recognizes the PL-binding site of β2GPI domain V.
J Thromb Haemost 1:740–747 Pengo V, Biasiolo A, Pegoraro C, Iliceto S (2004) A two-step coagulation test to identify antibeta-glycoprotein I lupus anticoagulants. J Thromb Haemost 2:702–707 de Laat B, Derksen RH, Urbanus RT, de Groot PG (2005) IgG antibodies that recognize epitope Gly40-Arg43 in domain I of beta 2-glycoprotein I cause LAC, and their presence correlates strongly with thrombosis. Blood 105:1540–1545 Thiagarajan P, Shapiro SS (1998) Lupus anticoagulants and antiphospholipid antibodies.
Pini, Pzza. C. it C. Crotti • C. Chighizola Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy Istituto G. L. L. Meroni et al. β2GPI-dependent aPL are more predictive for the vascular (and the obstetric) manifestations of the syndrome [2, 3]. aPL reacting with human β2GPI and cross-reacting with the animal molecule were shown to be pathogenic in all the in vivo experimental models. More importantly, the thrombogenic effect was reproduced by affinity-purified anti-β2GPI IgG and inhibited by specific absorption of the anti-β2GPI activity .