1/19. Renal involvement of thrombotic thrombocytopenic purpura: special reference to the glomeruloid structures.We report the case of a 9-year-old girl with biopsy-proven renal thrombotic microangiopathy in thrombotic thrombocytopenic purpura (TTP), with particular reference to the glomeruloid structures. The renal biopsy sample from this TTP patient revealed platelet thrombus deposition, a glomeruloid structure and aneurysm with relative sparing of the glomeruli. The glomeruloid structure displayed a proliferation of mainly capillary-sized channels lined by factor viii-related, antigen-positive plump endothelial cells embedded in the edematous connective tissue. These glomeruloid vessels communicated with the aneurysmal segment at the end portion of the arteriolar branch. We believe that the glomeruloid structures in TTP represent not merely organization or recanalization of thrombus but rather active angiogenesis through aneurysmal dilation in the arteriolized vessel, probably initiated by platelet agglutination.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/19. Autosomal recessive inheritance of von willebrand factor-cleaving protease deficiency.A child with chronic relapsing thrombotic thrombocytopenic purpura (TTP/HUS) had recurrent thrombocytopenia, microangiopathic hemolytic anemia with fragmented erythrocytes, microthrombi in the lung vessels, and renal dysfunction. Assay of von willebrand factor (vWF)-cleaving protease showed a complete protease deficiency in the patient and subnormal activities in the mother and in two asymptomatic siblings. No inhibitor of vWF-cleaving protease was detected in the patient's plasma. Periodic transfusions of fresh-frozen plasma prevented further acute episodes of TTP/HUS. Specific diagnosis of the constitutional deficiency of vWF-cleaving protease helps to provide successful prophylactic therapy.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
3/19. Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
4/19. Thrombotic thrombocytopenic purpura without any evidence of thrombotic lesions at autopsy.diagnosis of thrombotic thrombocytopenic purpura (TTP) is usually based upon the clinical features, and does not always involve histopathological evidence. We recently had experience with a patient who developed the five signs characteristic for TTP. He had been treated for liver cirrhosis associated with chronic hepatitis b infection, and the hepatic function was severely impaired at admission. Blood levels of vWF (von willebrand factor) and factor viii were highly elevated to 506% and 632%, respectively. These findings suggested severe endothelial damage. Thus, the patient was diagnosed as having TTP secondary to severe hepatic damage, and plasma exchanges were initiated immediately. He responded poorly to the treatment, and finally died of pulmonary hemorrhage. At autopsy, hepatocellular carcinoma was identified in the cirrhotic liver, but it was surprising that thorough postmortem examination failed to show any evidence of thrombotic lesions. Our experience suggests that secondary TTP does not always involve pathological evidence of the thrombotic lesions, and that the formation of thrombi causing vessel occlusion might not be essential in the pathogenesis of some secondary TTP.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/19. Intravascular lymphoma - a rare cause of hemolytic anemia and neurologic disorders.Intravascular lymphoma is an uncommon and often overlooked form of non-Hodgkin's lymphoma characterized by extensive proliferation of lymphoid cells within the lumina of small and medium-sized vessels. Clinical symptoms of the disease are variable and often nonspecific, mostly neurologic in nature. With an aggressive course, intravascular lymphomatosis has a poor prognosis and is rarely diagnosed ante mortem. We describe here a 76-year-old woman with the clinical diagnoses of hemolytic anemia and progressive lethargy where intravascular lymphomatosis turned out as the underlying cause of the disease.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
6/19. complement factor h mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement.Thrombotic thrombocytopenic purpura is a rare disorder of small vessels that is associated with deficiency of the von willebrand factor-cleaving protease ADAMTS13, which favors platelet adhesion and aggregation in the microcirculation. The disease manifests mainly with central nervous system symptoms, but cases of renal insufficiency have been reported. Presented are findings of the genetic basis of phenotype heterogeneity in thrombotic thrombocytopenic purpura in two sisters within one family. The patients had ADAMTS13 deficiency as a result of two heterozygous mutations (causing V88M and G1239V changes). In addition, a heterozygous mutation (causing an S890I change) in factor H of complement was found in the patient who developed chronic renal failure but not in her sister, who presented with exclusive neurologic symptoms.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
7/19. Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report.Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of small vessels that is associated with deficiency of the von willebrand factor-cleaving protease, ADAMTS13. The presence of anti-ADAMTS13 autoantibodies is considered a factor predisposing to relapses. Despite close monitoring and intensive plasma treatment, in these patients acute episodes are still associated with substantial morbidity and mortality rates, and the optimal therapeutic option should be prevention of relapses. This study was conducted in a patient with recurrent TTP due to high titers of ADAMTS13 inhibitors, who used to have 2 relapses of TTP a year. The study compared the standard treatment plasma exchange with rituximab. Results documented that plasma exchange had only a small transient effect on ADAMTS13 activity and inhibitors; on the contrary, prophylaxis with rituximab was associated with disappearance of anti-ADAMTS13 antibodies, a progressive recovery of protease activity, and it allowed the patient to maintain a disease-free state during a more than 2-year follow-up.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
8/19. Factors affecting the response to plasma therapy in thrombotic thrombocytopenic purpura.Factors affecting the response to plasma therapy (plasma exchange and plasma infusion) were studied in four cases of thrombotic thrombocytopenic purpura (TTP) treated in our hospital. Plasma therapy led to recovery from advanced TTP, but three of the patients exhibited chronic relapse. The longer the period from onset, the more marked was the progression of TTP, from the first stage (thrombocytopenia alone) to the second stage in which thrombocytopenia was accompanied by microangiopathic hemolytic anemia (MHA), and the third stage in which neurological abnormalities appeared. Platelet thrombi, which are thought to be the primary pathogenetic feature, probably enlarge leading to stenosis of the vessels during the progression of TTP. Although the severity of TTP in the early stages could be judged from two markers, LDH and the platelet count, it was impossible to determine the severity of advanced TTP by these two markers. However, the severity of advanced TTP could be judged by including the grade and period of neurological abnormalities. The dose of plasma needed to induce recovery was small in the early stage, but as the period from onset lengthened, the dose had to be increased. Though the early platelet thrombi might be easily eliminated by plasma infusion, larger and well-established platelet thrombi might not be lysed even by massive doses of plasma.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
9/19. Thrombotic thrombocytopenic purpura in a human immunodeficiency virus (hiv)-seropositive homosexual man.A case of thrombotic thrombocytopenic purpura (TTP) in a human immunodeficiency virus (hiv)-seropositive homosexual man is reported. The patient improved after corticosteroid and plasma exchange therapy was instituted. The case demonstrated most of the classic features of TTP, including histologic evidence of fibrin thrombi in small blood vessels. Atypical features included reduced number of megakaryocytes in the bone marrow and only partial resolution of thrombocytopenia after therapy was begun. An autoimmune thrombocytopenia has been well-characterized in hiv-antibody-seropositive homosexual men. physicians caring for hiv-seropositive persons should also be aware of a possible association with TTP.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
10/19. Thrombotic thrombocytopenic purpura with C'3 vascular deposits: report of a case.A case of thrombotic thrombocytopenic purpura in a young woman in which platelet microthrombi were distributed in small vessels throughout the body is reported. IgM and complement (C'3) were demonstrated in capillary and small arteriole walls by immunofluorescent microscopy. These findings support the findings of a previous case report, and are further evidence for a possible primary immune etiology for thrombotic thrombocytopenic purpura.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
| | Next -> |