1/26. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
2/26. Two cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies.We report 2 cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies. In the first case the titer of antiphospholipid antibodies was neither related to disease activity nor influenced by immunosuppressive therapy. In the second, cerebral infarction occurred in spite of severe thrombocytopenia. Our results provide additional evidence that antiphospholipid antibodies are not involved in the pathogenesis of autoimmune thrombocytopenic purpura. Nevertheless, the frequency and clinical significance of this association would suggest that patients with autoimmune thrombocytopenic purpura should be tested for antiphospholipid antibodies, particularly before pregnancy or surgical procedures.- - - - - - - - - - ranking = 7keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
3/26. Relapsing catastrophic antiphospholipid antibody syndrome: a mimic for thrombotic thrombocytopenic purpura?SUMMARY: The catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon disorder characterized by widespread micro- and macrovascular changes due to intravascular thrombosis. This complication of the antiphospholipid antibody syndrome is often fatal and recurrences are very rare. The differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP) and this distinction may be difficult, but essential, for appropriate therapy. plasmapheresis is effective in both conditions, but anticoagulation, a mainstay in the treatment of CAPS, could be disastrous in TTP. We present the case of an elderly woman who survived two episodes of CAPS four years apart and whose clinical findings were also suggestive of TTP. The characteristics of TTP and CAPS are compared and the importance of accurate diagnosis is emphasized.- - - - - - - - - - ranking = 5keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
4/26. 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 = thrombocytopenic, purpura (Clic here for more details about this article) |
5/26. splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
6/26. A role for transoesophageal echocardiography in the early diagnosis of catastrophic antiphospholipid syndrome.We describe a previously healthy 28-year-old woman who presented with the clinical picture of large vessel occlusions (stroke with left hemiparesis, myocardial infarction) and developed multi-organ failure (i.e. kidneys, heart, brain, liver, blood, skin) over a very short period of time. Peripheral blood smear was consistent with thrombotic thrombocytopenic purpura. Transesophageal echocardiogram was supportive of the diagnosis of catastrophic antiphospholipid syndrome (CAPS), revealing Libman-Sacks endocarditis. blood cultures were negative, anticardiolipin antibodies were highly increased and lupus anticoagulant was positive. Cerebral and coronary angiograms were negative, suggesting possible microthrombotic occlusive disease in the setting of CAPS.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
7/26. Primary antiphospholipid syndrome presenting as complicated Henoch-Schonlein purpura.A child showing signs of Henoch-Schonlein purpura developed a right tibiofibular vascular thrombosis. Antiphospholipid antibody tests were positive for both lupus anticoagulant and anticardiolipin antibodies. This suggests that an antiphospholipid syndrome should be considered in cases of Henoch-Schonlein purpura and antiphospholipid antibodies should be measured to determine whether prophylactic antithrombotic measures are needed to prevent thrombotic manifestations.- - - - - - - - - - ranking = 1.7466944957167keywords = purpura (Clic here for more details about this article) |
8/26. Antiphospholipid antibody syndrome complicated by Grave's disease.The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity.- - - - - - - - - - ranking = 0.29111574928611keywords = purpura (Clic here for more details about this article) |
9/26. Acute renal failure complicating hellp syndrome, SLE and anti-phospholipid syndrome: successful outcome using plasma exchange therapy.Renal disease is very common in patients with systemic lupus erythematosis (SLE) and it may emerge during pregnancy or the post-partum period. patients with anti-phospholipid syndrome (APS)are also at risk of renal disease. We present a case of acute renal failure in the post-partum period in a patient with SLE and APS. This case illustrates the potential difficulties in reaching a diagnosis in such a patient. It also illustrates the complexities of management and the potential interactions between SLE, Haemolysis, elevated liver enzymes and low platelets syndrome, thrombotic thrombocytopenic purpura, haemolytic uraemic syndrome and APS. We also review the role of plasma exchange therapy in managing our patient.- - - - - - - - - - ranking = 1keywords = thrombocytopenic, purpura (Clic here for more details about this article) |
10/26. Acute bullous purpura associated with hyperhomocysteinemia and antiphospholipid antibodies.We describe a female patient with an acute purpuric and bullous eruption mainly affecting the lower aspect of the legs. skin biopsy specimens demonstrated microvascular occlusions with fibrin thrombi but no dermal inflammation. Intermediate hyperhomocysteinemia and transient antiphospholipid antibodies were evidenced as factors of thrombophilia. The responsibility of the latter in the onset of the cutaneous lesions is discussed.- - - - - - - - - - ranking = 1.1644629971444keywords = purpura (Clic here for more details about this article) |
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