Journal of Perinatal Medicine
Vol.46, Issue4, P. 387-400
Аннотация:
Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called "thrombotic storm" and multiple organ failure, that is, CAPS. Since 2001-2016, we discovered 17 patients with CAPS development. CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30[%]-40[%]. © 2018 Walter de Gruyter GmbH, Berlin/Boston.
Ключевые слова:
Antiphospholipid syndrome; catastrophic APS; rare disease; thrombotic storm
acetylsalicylic acid; ancrod; anticoagulant agent; bemiparin; corticosteroid; cyclophosphamide; dalteparin; defibrotide; enoxaparin; fibrinolytic agent; fresh frozen plasma; glucocorticoid; heparin; hirudin; immunoglobulin; low molecular weight heparin; methylprednisolone; nadroparin; phospholipid antibody; prostacyclin; warfarin; anticoagulant therapy; antiphospholipid syndrome; Article; catastrophic antiphospholipid syndrome; chorioamnionitis; cytokine production; cytokine release; differential diagnosis; disseminated intravascular clotting; endothelial dysfunction; genetic predisposition; HELLP syndrome; heparin induced thrombocytopenia; human; microthrombus; multiple organ failure; nonhuman; pathophysiology; plasma exchange; preeclampsia; pregnancy complication; pregnant woman; priority journal; sepsis; solutio placentae; systemic inflammatory response syndrome; thrombophilia; thrombotic thrombocytopenic purpura; antiphospholipid syndrome; classification; complication; female; preg
Makatsariya A. D. Aleksandr Davidovich 1944-
Khizroeva D. Kh. Dzhamilya Khizrievna 1973-
Bitsadze V. O. Viktoriya Omarovna 1970-
Макацария А. Д. Александр Давидович 1944-
Хизроева Д. Х. Джамиля Хизриевна 1973-
Бицадзе В. О. Виктория Омаровна 1970-
Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology
Текст визуальный непосредственный
Journal of Perinatal Medicine
Walter de Gruyter GmbH
Vol.46, Issue4 P. 387-400
2018
Статья
Antiphospholipid syndrome catastrophic APS rare disease thrombotic storm
acetylsalicylic acid ancrod anticoagulant agent bemiparin corticosteroid cyclophosphamide dalteparin defibrotide enoxaparin fibrinolytic agent fresh frozen plasma glucocorticoid heparin hirudin immunoglobulin low molecular weight heparin methylprednisolone nadroparin phospholipid antibody prostacyclin warfarin anticoagulant therapy antiphospholipid syndrome Article catastrophic antiphospholipid syndrome chorioamnionitis cytokine production cytokine release differential diagnosis disseminated intravascular clotting endothelial dysfunction genetic predisposition HELLP syndrome heparin induced thrombocytopenia human microthrombus multiple organ failure nonhuman pathophysiology plasma exchange preeclampsia pregnancy complication pregnant woman priority journal sepsis solutio placentae systemic inflammatory response syndrome thrombophilia thrombotic thrombocytopenic purpura antiphospholipid syndrome classification complication female preg
Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called "thrombotic storm" and multiple organ failure, that is, CAPS. Since 2001-2016, we discovered 17 patients with CAPS development. CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30[%]-40[%]. © 2018 Walter de Gruyter GmbH, Berlin/Boston.