1/514. thrombotic microangiopathies and hiv infection: report of two typical cases, features of HUS and TTP, and review of the literature.Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies increasingly reported in patients with hiv infection. However, characteristic features of thrombotic microangiopathies associated with hiv disease have not been defined yet. The typical courses of HUS and TTP in two patients are presented. The data as well as the analysis of cases published in the literature demonstrate the association of thrombotic microangiopathies with late-stage hiv disease. Moreover, differences between HUS and TTP can be detected. patients with HUS present with more severe immunologic deterioration. Although clinical symptoms are fewer, HUS implicates a very poor prognosis. life expectancy rarely exceeded 1 year after diagnosis. HUS and TTP should therefore be added to the international AIDS classification.- - - - - - - - - - ranking = 1keywords = purpura (Clic here for more details about this article) |
2/514. A case of thrombotic thrombocytopenic purpura in an adult treated with vincristine.The case of a woman with thrombotic thrombocytopenic purpura refractory to prolonged treatment with plasma exchange and steroid treatment is described. The addition of vincristine yielded a complete response, which has been maintained for 9 months up to the time of this report.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
3/514. Thrombotic thrombocytopenic purpura: brain CT and MRI findings in 12 patients.Clinical-neuroimaging analysis of 12 thrombotic thrombocytopenic purpura (TTP) patients revealed a variety of brain lesions. These included reversible cerebral edema lesions with MRI features of reversible posterior leukoencephalopathy syndrome (RPLS). Most of the RPLS patients had hypertension and renal dysfunction, suggesting RPLS due to hypertensive encephalopathy. Prompt treatment usually led to neurologic recovery and disappearance of edematous lesions. Those with infarcts or hematomas had a poorer outcome. TTP should be added to the expanding spectrum of RPLS and hypertensive encephalopathy.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
4/514. An uncommon case of late thrombotic thrombocytopenic purpura (42 months) after autologous peripheral blood stem cell (PBSC) transplantation in a child.Thrombotic thrombocytopenic purpura (TTP) is a serious complication following bone marrow transplantation (BMT). Therapeutic plasma exchange does not appear to be effective in its management in contrast to classical TTP. We report the case of a child with TTP 3 years after autologous PBSC transplantation, in whom remission was achieved by administration of cyclosporin A, after failure of plasmapheresis as primary treatment.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
5/514. 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 = purpura (Clic here for more details about this article) |
6/514. Solvent/detergent fresh frozen plasma as primary treatment of acute thrombotic thrombocytopenic purpura.Three patients with acute thrombotic thrombocytopenic purpura (TTP) (one associated with E. coli infection) received as primary treatment pooled fresh frozen plasma (FFP) virus inactivated by the solvent/detergent (SD) method, plus prednisolone. All three patients attained platelet counts of > 50 x 10(9)/l by days 7, 3 and 10, respectively. serum creatinine became corrected in parallel. Neurological features were slow to resolve in one patient, and developed de novo during plasma infusion in another with rapid reversal once intensive plasma exchange with SDFFP was instituted. All patients are in stable remission > 1 years later. SDFFP was well tolerated with no febrile or other reactions, and all patients remained negative for markers of infection with hiv, hepatitis b and hepatitis c viruses. One patient was positive for antibodies for hepatitis a virus and parvovirus B19 prior to treatment. The other two patients remained antibody negative at the end of therapy.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
7/514. Cancer chemotherapy-related thrombotic thrombocytopenic purpura: biological evidence of increased nitric oxide production.The occurrence of thrombotic thrombocytopenic purpura (TTP) in cancer patients receiving chemotherapy has been well established; although this entity is rare, its clinical importance seems to be growing. We describe 3 cases of TTP developing in cancer patients receiving different chemotherapeutic regimens. Using a sensitive high-performance liquid chromatographic method, we evaluated the stable nitric oxide end products, nitrite and nitrate, in the plasma of these patients. nitric oxide is one of the key components involved in maintaining the normal nonthrombogenicity of the vascular endothelium. In our 3 patients, we found increased nitrate titers that were substantially higher than those observed in patients with de novo TTP. The observed increased release of nitrate could be interpreted as the consequence of massive disruption of endothelial integrity, with consequent passive nitric oxide release in vivo, or an adaptive mechanism of the endothelium to compensate for diffuse microvascular occlusion. The 2 mechanisms may both be involved, but the normal titers of nitric oxide end products in de novo TTP suggest that the former mechanism is more important, at least in cancer chemotherapy-related TTP.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
8/514. Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
9/514. Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy.maternal death during pregnancy, although uncommon, may result from a broad range of conditions. In this paper, a case of thrombotic thrombocytopenic purpura diagnosed by postmortem examination is presented. Thrombotic thrombocytopenic purpura is one of a subset of diseases that result in the formation of microthrombi within the vasculature, either as a primary or secondary manifestation. Other conditions included in the differential diagnosis during pregnancy are hemolytic uremic syndrome, systemic lupus erythematosus, preeclampsia-eclampsia and the hellp syndrome, acute fatty liver of pregnancy, antiphospholipid antibody syndrome, and disseminated intravascular coagulation. The histologic manifestations of these diseases can be similar and in most cases do not provide adequate information to accurately differentiate these diseases in the postmortem period. This paper addresses the need for clinical history (i.e., symptomatology, trimester of onset) and antemortem laboratory testing in addition to a thorough autopsy to accurately differentiate among the conditions named previously. In the absence of an adequate clinical history and antemortem laboratory testing, the more general diagnosis of "thrombotic microangiopathy of pregnancy" is acceptable.- - - - - - - - - - ranking = 6keywords = purpura (Clic here for more details about this article) |
10/514. Thrombotic thrombocytopenic purpura today.Thrombotic thrombocytopenic purpura (TTP) usually responds to plasma exchange and plasma infusion. However, until recently, it was not clear how the treatment works. New understanding of the pathophysiology of TTP has clarified that issue and suggested new therapies that may eliminate plasma infusion and its risks.- - - - - - - - - - ranking = 5keywords = purpura (Clic here for more details about this article) |
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