1/8. Catastrophic antiphospholipid syndrome associated with crescentic glomerulonephritis: a clinicopathologic case.The association of renal failure with catastrophic antiphospholipid syndrome has been reported in the context of microvascular occlusions and/or malignant hypertension. We describe a 36-year-old woman who died of multiorgan failure with the laboratory, clinical and histopathological characteristics of catastrophic antiphospholipid syndrome associated with a crescentic glomerulonephritis and renal failure.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/8. Catastrophic antiphospholipid antibody syndrome in systemic lupus erythematosus: an autopsy case report of a young woman.Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome (APS) characterized by disseminated microangiopathy that results in multiorgan failure. CAPS mainly occurs in association with systemic lupus erythematosus (SLE). Clinically, CAPS mimics disseminated SLE vasculitis, intravascular coagulation (DIC), and particularly thrombotic thrombocytopenic purpura (TTP). We describe an autopsy case of young woman with CAPS in SLE, which is difficult to differentiate from TTP secondary to SLE.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
3/8. Childhood acute myelomonocytic leukemia (AML-M4) presenting as catastrophic antiphospholipid antibody syndrome.: The authors describe a 15-year-old girl presenting with a cerebral ischemic stroke as the first manifestation of catastrophic antiphospholipid antibody syndrome secondary to acute myeloid leukemia (AML). Despite treatment with anticoagulants, therapeutic plasma exchange, and chemotherapy, the patient developed multiorgan thromboses and failure, eventually culminating in death. This unusual presentation of AML has not been previously described in children. Clinical features of antiphospholipid antibody syndrome and current knowledge regarding its association with malignancies are reviewed.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/8. Catastrophic antiphospholipid syndrome; a "CATASTROPHIC" case of systemic lupus erythematosus.Less than 1% of patients with the antiphospholipid antibody syndrome (APS) can develop multiple, simultaneous organ-system thromboembolic disease, which is referred to as the catastrophic antiphospholipid antibody syndrome (CAPS). Roughly one-half of these patients have systemic lupus erythematosus (SLE). Factors known to precipitate CAPS include infection, surgery, trauma, neoplasia, anticoagulation withdrawal, obstetric complications, and SLE flares. Optimal treatment includes anticoagulation and high-dose corticosteroids, although IVIG and plasma exchange may also have a role. The overall mortality of CAPS is roughly 50%, but prompt clinical recognition of this rare but devastating syndrome may lead to improved outcomes.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/8. Catastrophic antiphospholipid syndrome in a 14-year-old child.antiphospholipid syndrome (APS) is an autoimmune disease. Less than 1% of patients with APS present with life-threatening catastrophic APS (CAPS). We report here a case of CAPS in a young girl with cardiac, gastrointestinal and renal involvement. Although the management was complicated, the outcome was better than expected. We suggest that CAPS be included in the differential diagnosis of acute renal failure in children with multi-organ involvement and prolonged phospholipid-dependent coagulation time and promptly treated with immunomodulating agents and anticoagulants.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/8. Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury - a case report with pathological and molecular correlative studies.Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare variant of antiphospholipid syndrome (APS) characterized by acute multiorgan failure due to small vessel thrombi in patients with positive antiphospholipid antibodies. We report a fatal case of catastrophic antiphospholipid syndrome in a young woman with a history of polymyositis and Hodgkin lymphoma. The patient was admitted to hospital because of severe foot pain following several weeks of skin ulcerations. Doppler ultrasonography showed evidence of arterial ischemia of the both lower extremities. Despite anticoagulation, immunosuppression, plasmapheresis and antibiotic therapy, she developed cutaneous gangrene, retroperitoneal hematoma, ileus, and acute respiratory and renal failure that resulted in death. autopsy showed multifocal vascular injury and microthrombi with associated hemorrhages and infarcts in multiple organs. The patient had normal levels of functional protein c and protein s and a normal level of plasma homocysteine. Tests for common thromophilic gene mutations including prothrombin 20210, factor v Leiden 1691, and methylene tetrahydrofolate reductase 677 were negative. To our knowledge, this is the first CAPS patient with molecular studies for genetic prothrombotic mutations. Our report showed that there was no association between the development of CAPS and inherited thromophilia.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
7/8. Catastrophic antiphospholipid antibody syndrome.Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/8. Catastrophic thromboses and severe thrombocytopenia during heparin therapy in a patient with anti-phospholipid syndrome.Catastrophic anti-phospholipid syndrome (CAPS) is a medical emergency characterized by thromboses of multiple small vessels of internal organs and the brain. Herein we present a patient with primary anti-phospholipid syndrome who developed CAPS manifested by hepatic, renal and splenic artery thromboses, as well as cerebral venous thrombosis. The course was further complicated by severe thrombocytopenia and haemolytic anemia. Two episodes of catastrophic thrombosis developed within 24-36 h after the initiation of heparin therapy, suggesting a role of heparin in triggering thromboses. The patient had no anti-platelet-factor-4 antibodies in repeated measurements, making clinical diagnosis of heparin-induced thrombocytopenia unlikely. The possible role of heparin in induction of thromboses and its therapeutic implication are detailed.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |