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Case Rep Med
August 2012
Department of Obstetrics and Gynecology, Nagoya City University, Graduate School of Medicine, Nagoya 467-8601, Japan.
Microangiopathic antiphospholipid-associated syndromes (MAPSs) are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet); kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura). It is predominant in patients with catastrophic antiphospholipid syndrome (APS). A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients.
View Article and Find Full Text PDFLupus
September 2008
Department of Nephrology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia.
A 4-year-old boy with an atypical course of haemolytic uremic syndrome (HUS), who developed microangiopathic antiphospholipid-associated syndrome (MAPS) with signs of multiple organ failure during the course of his disease, is reported. Early and aggressive treatment with intravenous gammaglobulin, pulse methylprednisolone and plasmapheresis resulted in an excellent clinical recovery. Our patient showed a concomitant presence of multiple factors that could precipitate atypical HUS, including positive antiphospholipid antibodies, decreased level of factor H and positive anti-ADAMTS-13 antibodies.
View Article and Find Full Text PDFSemin Thromb Hemost
April 2008
Division of Immunology, The School of Pathology, University of the Witwatersrand, The Netcare Rosebank Hospital, Rosebank, Johannesburg, South Africa.
This article provides a review of the various types of antiphospholipid (aPL) antibodies and antiphospholipid syndromes, their prevalence, presumed origin, relationship to autoimmunity in general, and their role in the body's defenses and apoptosis. New hypotheses such as the role of antibodies to beta2 glycoprotein I (beta2GPI) and the signaling of toll-like receptors are also discussed, as is the spectrum of clinical manifestations associated with the demonstration of these antibodies, now assumed to be "pathogenic." A distinction is made between antibodies present in sera of patients with a variety of microangiopathic syndromes (MAPS; e.
View Article and Find Full Text PDFAutoimmun Rev
January 2008
Division of Immunology, School of Pathology, University of the Witwatersrand the Netcare Rosebank Hospital, Johannesburg, South Africa.
Small vessel occlusions may occur as part of the vascular manifestations of the Antiphospholipid Syndrome (APS) and may affect glomerular, skin, retinal, bowel, hepatic or pulmonary vessels. These thrombotic lesions are proven (usually by biopsy, surgical procedures, at autopsy or by specialized techniques e.g.
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